EX-3.(II)

AMENDED AND RESTATED BYLAWS

OF

BIOMARIN PHARMACEUTICAL INC.

ARTICLE I

CORPORATE OFFICES

1.1 REGISTERED OFFICE

        The registered office of the Corporation shall be in the City of Wilmington, County of New Castle, State of Delaware. The name of the registered agent of the Corporation at such location is The Corporation Trust Company.

1.2   OTHER OFFICES

        The Board of Directors may at any time establish other offices at any place or places where the Corporation is qualified to do business.

ARTICLE II

MEETINGS OF STOCKHOLDERS

2.1   PLACE OF MEETINGS

        Meetings of stockholders shall be held at any place, within or outside the State of Delaware, designated by the Board of Directors. In the absence of any such designation, stockholders’ meetings shall be held at the registered office of the Corporation.

2.2   ANNUAL MEETING

        The annual meeting of stockholders shall be held each year on a date and at a time designated by the Board of Directors. In the absence of such designation, the annual meeting of stockholders shall be held on the third Wednesday of June in each year at 10 a.m. However, if such day falls on a legal holiday, then the meeting shall be held at the same time and place on the next succeeding full business day. At the meeting, directors shall be elected and any other proper business may be transacted.

2.3   SPECIAL MEETING

        A special meeting of the stockholders may be called, at any time for any purpose or purposes, by the Board of Directors or by such person or persons as may be authorized by the Certificate of Incorporation or the Bylaws.

        Upon the effective date of the final prospectus in connection with the initial public offering of the Corporation’s capital stock, a special meeting of the stockholders may be called at any time for any purpose or purposes by the Chairman of the Board of Directors or by a majority of the then current members of the Board of Directors.

2.4   NOTICE OF STOCKHOLDERS’ MEETINGS

        All notices of meetings with stockholders shall be in writing and shall be sent or otherwise given in accordance with Section 2.5 of these bylaws not less than ten (10) nor more than sixty (60) days before the date of the meeting to each stockholder entitled to vote at such meeting. The notice shall specify the place, date, and hour of the meeting, and, in the case of a special meeting, the purpose or purposes for which the meeting is called.

2.5   MANNER OF GIVING NOTICE; AFFIDAVIT OF NOTICE

        Written notice of any meeting of stockholders, if mailed, is given when deposited in the United States mail, postage prepaid, directed to the stockholder at his address as it appears on the records of the Corporation. An affidavit of the secretary or an assistant secretary or of the transfer agent of the Corporation that the notice has been given shall, in the absence of fraud, be prima facie evidence of the facts stated therein.

2.6   QUORUM

        The holders of a majority of the stock issued and outstanding and entitled to vote thereat, present in person or represented by proxy, shall constitute a quorum at all meetings of the stockholders for the transaction of business except as otherwise provided by statute or by the certificate of incorporation. If, however, such quorum is not present or represented at any meeting of the stockholders, then the stockholders entitled to vote thereat, present in person or represented by proxy, shall have power to adjourn the meeting from time to time, without notice other than announcement at the meeting, until a quorum is present or represented. At such adjourned meeting at which a quorum is present or represented, any business may be transacted that might have been transacted at the meeting as originally noticed.

2.7   ADJOURNED MEETING; NOTICE

        When a meeting is adjourned to another time or place, unless these bylaws otherwise require, notice need not be given of the adjourned meeting if the time and place thereof are announced at the meeting at which the adjournment is taken. At the adjourned meeting the Corporation may transact any business that might have been transacted at the original meeting. If the adjournment is for more than thirty (30) days, or if after the adjournment a new record date is fixed for the adjourned meeting, a notice of the adjourned meeting shall be given to each stockholder of record entitled to vote at the meeting.

2.8   VOTING

        The stockholders entitled to vote at any meeting of stockholders shall be determined in accordance with the provisions of Section 2.11 of these bylaws, subject to the provisions of Sections 217 and 218 of the General Corporation Law of Delaware (relating to voting rights of fiduciaries, pledgors and joint owners of stock and to voting trusts and other voting agreements).

        Except as may be otherwise provided in the certificate of incorporation, each stockholder shall be entitled to one vote for each share of capital stock held by such stockholder.

2.9   WAIVER OF NOTICE

        Whenever notice is required to be given under any provision of the General Corporation Law of Delaware or of the certificate of incorporation or these bylaws, a written waiver thereof, signed by the person entitled to notice, whether before or after the time stated therein, shall be deemed equivalent to notice. Attendance of a person at a meeting shall constitute a waiver of notice of such meeting, except when the person attends a meeting for the express purpose of objecting, at the beginning of the meeting, to the transaction of any business because the meeting is not lawfully called or convened. Neither the business to be transacted at, nor the purpose of, any regular or special meeting of the stockholders need be specified in any written waiver of notice unless so required by the certificate, of incorporation or these bylaws.

2.10  STOCKHOLDER ACTION BY WRITTEN CONSENT WITHOUT A MEETING

        Unless otherwise provided in the certificate of incorporation, any action required by this chapter to be taken at any annual or special meeting of stockholders of the Corporation, or any action that may be taken at any annual or special meeting of such stockholders, may be taken without a meeting, without prior notice, and without a vote if a consent in writing, setting forth the action so taken, is signed by the holders of outstanding stock having not less than the minimum number of votes that would be necessary to authorize or take such action at a meeting at which all shares entitled to vote thereon were present and voted.

        Prompt notice of the taking of the corporate action without a meeting by less than unanimous written consent shall be given to those stockholders who have not consented in writing. If the action which is consented to is such as would have required the filing of a certificate under any section of the General Corporation Law of Delaware if such action had been voted on by stockholders at a meeting thereof, then the certificate filed under such section shall state, in lieu of any statement required by such section concerning any vote of stockholders, that written notice and written consent have been given as provided in Section 228 of the General Corporation Law of Delaware.

        Upon the effective date of the final prospectus in connection with the initial public offering of any of the Corporation’s securities, the stockholders of the Corporation may not take any action by written consent without a meeting but must take any such action at a duly called annual or special meeting of stockholders.

2.11  RECORD DATE FOR STOCKHOLDER NOTICE; VOTING GIVING CONSENTS

        In order that the Corporation may determine the stockholders entitled to notice of or to vote at any meeting of stockholders or any adjournment thereof, or entitled to express consent to corporate action in writing without a meeting, or entitled to receive payment of any dividend or other distribution or allotment of any rights, or entitled to exercise any rights in respect of any change, conversion or exchange of stock or for the purpose of any other lawful action, the Board of Directors may fix, in advance, a record date, which shall not be more than sixty (60) nor less than ten (10) days before the date of such meeting, nor more than sixty (60) days prior to any other action.

If the Board of Directors does not so fix a record date:

(i)   The record date for determining stockholders entitled to notice of or to vote at a meeting of stockholders shall be at the close of business on the day next preceding the day on which notice is given, or, if notice is waived, at the close of business on the day next preceding the day on which the meeting is held.

(ii)  The record date for determining stockholders entitled to express consent to corporate action in writing without a meeting, when no prior action by the Board of Directors is necessary, shall be the day on which the first written consent is expressed.

(iii)  The record date for determining stockholders for any other purpose shall be at the close of business on the day on which the Board adopts the resolution relating thereto.

        A determination of stockholders of record entitled to notice of or to vote at a meeting of stockholders shall apply to any adjournment of the meeting; provided, however, that the Board of Directors may fix a new record date for the adjourned meeting.

2.12  PROXIES

        Each stockholder entitled to vote at a meeting of stockholders or to express consent or dissent to corporate action in writing without a meeting may authorize another person or persons to act for him by a written proxy, signed by the stockholder and filed with the secretary of the Corporation, but no such proxy shall be voted or acted upon after three (3) years from its date, unless the proxy provides for a longer period. A proxy shall be deemed signed if the stockholder’s name is placed on the proxy (whether by manual signature, typewriting, telegraphic transmission or otherwise) by the stockholder or the stockholder’s attorney-in-fact. The revocability of a proxy that states on its face that it is irrevocable shall be governed by the provisions of Section 212(c) of the General Corporation Law of Delaware.

2.13  LIST OF STOCKHOLDERS ENTITLED TO VOTE

        The officer who has charge of the stock ledger of the Corporation shall prepare and make, at least ten (10) days before every meeting of stockholders, a complete list of the stockholders entitled to vote at the meeting, arranged in alphabetical order, and showing the address of each stockholder and the number of shares registered in the name of each stockholder. Such list shall be open to the examination of any stockholder, for any purpose germane to the meeting, during ordinary business hours, for a period of at least ten (10) days prior to the meeting, either at a place within the city where the meeting is to be held, which place shall be specified in the notice of the meeting, or, if not so specified, at the place where the meeting is to be held. The list shall also be produced and kept at the time and place of the meeting during the whole time thereof, and may be inspected by any stockholder who is present.

ARTICLE III

DIRECTORS

3.1   POWERS

        Subject to the provisions of the General Corporation Law of Delaware and any limitations in the certificate of incorporation or these bylaws relating to action required to be approved by the stockholders or by the outstanding shares, the business and affairs of the Corporation shall be managed and all corporate powers shall be exercised by or under the direction of the Board of Directors.

3.2   NUMBER OF DIRECTORS

        The authorized number of directors shall be five (5). This number may be changed by a duly adopted amendment to the certificate of incorporation or by an amendment to this bylaw adopted by the vote or written consent of the holders of a majority of the stock issued and outstanding and entitled to vote or by resolution of a majority of the Board of Directors.

        No reduction of the authorized number of directors shall have the effect of removing any director before that director’s term of office expires.

3.3   ELECTION QUALIFICATION AND TERM OF OFFICE OF DIRECTORS

        Except as provided in Section 3.4 of these bylaws, directors shall be elected at each annual meeting of stockholders to hold office until the next annual meeting. Directors need not be stockholders unless so required by the certificate of incorporation or these bylaws, wherein other qualifications for directors may be prescribed. Each director, including a director elected to fill a vacancy, shall hold office until his successor is elected and qualified or until his earlier resignation or removal.

Elections of directors need not be by written ballot.

3.4   RESIGNATION AND VACANCIES

        Any director may resign at any time upon written notice to the Corporation. When one or more directors so resigns and the resignation is effective at a future date, a majority of the directors then in office, including those who have so resigned, shall have power to fill such vacancy or vacancies, the vote thereon to take effect when such resignation or resignations shall become effective, and each director so chosen shall hold office as provided in this section in the filling of other vacancies.

Unless otherwise provided in the certificate of incorporation or these bylaws:

(i)    Vacancies and newly created directorships resulting from any increase in the authorized number of directors elected by all of the stockholders having the right to vote as a single class may be filled by a majority of the directors then in office, although less than a quorum, or by a sole remaining director.

(ii)   Whenever the holders of any class or classes of stock or series thereof are entitled to elect one or more directors by the provisions of the certificate of incorporation, vacancies and newly created directorships of such class or classes or series may be filled by a majority of the directors elected by such class or classes or series thereof then in office, or by a sole remaining director so elected.

        If at any time, by reason of death or resignation or other cause, the Corporation should have no directors in office, then any officer or any stockholder or an executor, administrator, trustee or guardian of a stockholder, or other fiduciary entrusted with like responsibility for the person or estate of a stockholder, may call a special meeting of stockholders in accordance with the provisions of the certificate of incorporation or these bylaws, or may apply to the Court of Chancery for a decree summarily ordering an election as provided in Section 211 of the General Corporation Law of Delaware.

        If, at the time of filling any vacancy or any newly created directorship, the directors then in office constitute less than a majority of the whole Board (as constituted immediately prior to any such increase), then the Court of Chancery may, upon application of any stockholder or stockholders holding at least ten (10) percent of the total number of the shares at the time outstanding having the right to vote for such directors, summarily order an election to be held to fill any such vacancies or newly created directorships, or to replace the directors chosen by the directors then in office as aforesaid, which election shall be governed by the provisions of Section 211 of the General Corporation Law of Delaware as far as applicable.

3.5   PLACE OF MEETINGS; MEETINGS BY TELEPHONE

        The Board of Directors of the Corporation may hold meetings, both regular and special, either within or outside the State of Delaware.

        Unless otherwise restricted by the certificate of incorporation or these bylaws, members of the Board of Directors, or any committee designated by the Board of Directors, may participate in a meeting of the Board of Directors, or any committee, by means of conference telephone or similar communications equipment by means of which all persons participating in the meeting can hear each other, and such participation in a meeting shall constitute presence in person at the meeting.

3.6   FIRST MEETINGS

        The first meeting of each newly elected Board of Directors shall be held at such time and place as shall be fixed by the vote of the stockholders at the annual meeting and no notice of such meeting shall be necessary to the newly elected directors in order legally to constitute the meeting, provided a quorum shall be present. In the event of the failure of the stockholders to fix the time or place of such first meeting of the newly elected Board of Directors, or in the event such meeting is not held at the time and place so fixed by the stockholders, the meeting may be held at such time and place as shall be specified in a notice given as hereinafter provided for special meetings of the Board of Directors, or as shall be specified in a written waiver signed by all of the directors.

3.7   REGULAR MEETINGS

        Regular meetings of the Board of Directors may be held without notice at such time and at such place as shall from time to time be determined by the Board.

3.8   SPECIAL MEETINGS; NOTICE

        Special meetings of the Board of Directors for any purpose or purposes may be called at any time by the chairman of the Board, the president, any vice president, the secretary or any two (2) directors.

        Notice of the time and place of special meetings shall be delivered personally or by telephone to each director or sent by first-class mail or telegram, charges prepaid, addressed to each director at that director’s address as it is shown on the records of the Corporation. If the notice is mailed, it shall be deposited in the United States mail at least four (4) days before the time of the holding of the meeting. If the notice is delivered personally or by telephone or by telegram or by facsimile, it shall be delivered personally or by telephone or to the telegraph company at least one (1) hour before the time of the holding of the meeting. Any oral notice given personally or by telephone may be communicated either to the director or to a person at the office of the director who the person giving the notice has reason to believe will promptly communicate it to the director. The notice need not specify the purpose or the place of the meeting, if the meeting is to be held at the principal executive office of the Corporation.

3.9   QUORUM

        At all meetings of the Board of Directors, a majority of the authorized number of directors shall constitute a quorum for the transaction of business and the act of a majority of the directors present at any meeting at which there is a quorum shall be the act of the Board of Directors, except as may be otherwise specifically provided by statute or by the certificate of incorporation. If a quorum is not present at any meeting of the Board of Directors, then the directors present thereat may adjourn the meeting from time to time, without notice other than announcement at the meeting, until a quorum is present.

3.10  WAIVER OF NOTICE

        Whenever notice is required to be given under any provision of the General Corporation Law of Delaware or of the certificate of incorporation or these bylaws, a written waiver thereof, signed by the person entitled to notice, whether before or after the time stated therein, shall be deemed equivalent to notice. Attendance of a person at a meeting shall constitute a waiver of notice of such meeting, except when the person attends a meeting for the express purpose of objecting, at the beginning of the meeting, to the transaction of any business because the meeting is not lawfully called or convened. Neither the business to be transacted at, nor the purpose of, any regular or special meeting of the directors, or members of a committee of directors, need be specified in any written waiver of notice unless so required by the certificate of incorporation or these bylaws.

3.11  ADJOURNED MEETING: NOTICE

        If a quorum is not present at any meeting of the Board of Directors, then the directors present thereat may adjourn the meeting from time to time, without notice other than announcement at the meeting, until a quorum is present.

3.12  BOARD ACTION BY WRITTEN CONSENT WITHOUT A MEETING

        Unless otherwise restricted by the certificate of incorporation or these bylaws, any action required or permitted to be taken at any meeting of the Board of Directors, or of any committee thereof, may be taken without a meeting if all members of the Board or committee, as the case may be, consent thereto in writing and the writing or writings are filed with the minutes of proceedings of the Board or committee.

3.13  FEES AND COMPENSATION OF DIRECTORS

        Unless otherwise restricted by the certificate of incorporation or these bylaws, the Board of Directors shall have the authority to fix the compensation of directors.

3.14  APPROVAL OF LOANS TO OFFICERS

        The Corporation may lend money to, or guarantee any obligation of, or otherwise assist any officer or other employee of the Corporation or of its subsidiary, including any officer or employee who is a director of the Corporation or its subsidiary, whenever, in the judgment of the directors, such loan, guaranty or assistance may reasonably be expected to benefit the Corporation. The loan, guaranty or other assistance may be with or without interest and may be unsecured, or secured in such manner as the Board of Directors shall approve, including, without limitation, a pledge of shares of stock of the Corporation. Nothing in this section contained shall be deemed to deny, limit or restrict the powers of guaranty or warranty of the Corporation at common law or under any statute.

3.15  REMOVAL OF DIRECTORS

        Unless otherwise restricted by statute, by the certificate of incorporation or by these bylaws, any director or the entire Board of Directors may be removed, with or without cause, by the holders of a majority of the shares then entitled to vote at an election of directors.

        No reduction of the authorized number of directors shall have the effect of removing any director prior to the expiration of such director’s term of office.

ARTICLE IV

COMMITTEES

4.1   COMMITTEES OF DIRECTORS

        The Board of Directors may, by resolution passed by a majority of the whole Board, designate one or more committees, with each committee to consist of one or more of the directors of the Corporation. The Board may designate one or more directors as alternate members of any committee, who may replace any absent or disqualified member at any meeting of the committee. In the absence or disqualification of a member of a committee, the member or members thereof present at any meeting and not disqualified from voting, whether or not he or they constitute a quorum, may unanimously appoint another member of the Board of Directors to act at the meeting in the place of any such absent or disqualified member. Any such committee, to the extent provided in the resolution of the Board of Directors or in the bylaws of the Corporation, shall have and may exercise all the powers and authority of the Board of Directors in the management of the business and affairs of the Corporation, and may authorize the seal of the Corporation to be affixed to all papers that may require it; but no such committee shall have the power or authority to (i) amend the certificate of incorporation (except that a committee may, to the extent authorized in the resolution or resolutions providing for the issuance of shares of stock adopted by the Board of Directors as provided in Section 151 (a) of the General Corporation Law of Delaware) fix any of the preferences or rights of such shares relating to dividends, redemption, dissolution, any distribution of assets of the Corporation or the conversion into, or the exchange of such shares for, shares of any other class or classes or any other series of the same or any other class or classes of stock of the Corporation, (ii) adopt an agreement of merger or consolidation under Sections 251 or 252 of the General Corporation Law of Delaware, (iii) recommend to the stockholders the sale, lease or exchange of all or substantially all of the Corporation’s property and assets, (iv) recommend to the stockholders a dissolution of the Corporation or a revocation of a dissolution, or (v) amend the bylaws of the Corporation; and, unless the Board resolution establishing the committee, the bylaws or the certificate of incorporation expressly so provide, no such committee shall have the power or authority to declare a dividend, to authorize the issuance of stock, or to adopt a certificate of ownership and merger pursuant to Section 253 of the General Corporation Law of Delaware.

4.2   COMMITTEE MINUTES

        Each committee shall keep regular minutes of its meetings and report the same to the Board of Directors when required.

4.3   MEETINGS AND ACTION OF COMMITTEES

        Meetings and actions of committees shall be governed by, and held and taken in accordance, with, the provisions of Article III of these bylaws, Section 3.5 (place of meetings and meetings by telephone), Section 3.7 (regular meetings), Section 3.8 (special meetings and notice), Section 3.9 (quorum), Section 3.10 (waiver of notice), Section 3.11 (adjournment and notice of adjournment), and Section 3.12 (action without a meeting), with such changes in the context of those bylaws as are necessary to substitute the committee and its members for the Board of Directors and its members; provided, however, that the time of regular meetings of committees may also be called by resolution of the Board of Directors and that notice of special meetings of committees shall also be given to all alternate members, who shall have the right to attend all meetings of the committee. The Board of Directors may adopt rules for the government of any committee not inconsistent with the provisions of these bylaws.

ARTICLE V

OFFICERS

5.1   OFFICERS

        The officers of the Corporation shall be a president, one or more vice presidents, a secretary, and a treasurer. The Corporation may also have, at the discretion of the Board of Directors, a chairman of the Board, one or more assistant vice presidents, assistant secretaries, assistant treasurers, and any such other officers as may be appointed in accordance with the provisions of Section 5.3 of these bylaws. Any number of offices may be held by the same person.

5.2   ELECTION OF OFFICERS

        The officers of the Corporation, except such officers as may be appointed in accordance with the provisions of Sections 5.3 or 5.5 of these bylaws, shall be chosen by the Board of Directors, subject to the rights, if any, of an officer under any contract of employment.

5.3   SUBORDINATE OFFICERS

        The Board of Directors may appoint, or empower the president (or if there is no president, the chief executive officer) to appoint such other officers and agents as the business of the Corporation may require, each of whom shall hold office for such period, have such authority, and perform such duties as are provided in these bylaws or as the Board of Directors may from time to time determine.

5.4   REMOVAL AND RESIGNATION OF OFFICERS

        Subject to the rights, if any, of an officer under any contract of employment, any officer may be removed, either with or without cause, by an affirmative vote of the majority of the Board of Directors at any regular or special meeting of the Board or, except in the case of an officer chosen by the Board of Directors, by any officer upon whom such power of removal may be conferred by the Board of Directors.

        Any officer may resign at any time by giving written notice to the Corporation. Any resignation shall take effect at the date of the receipt of that notice or at any later time specified in that notice; and, unless otherwise specified in that notice, the acceptance of the resignation shall not be necessary to make it effective. Any resignation is without prejudice to the rights, if any, of the Corporation under any contract to which the officer is a party.

5.5   VACANCIES IN OFFICES

        Any vacancy occurring in any office of the Corporation shall be filled by the Board of Directors.

5.6   CHAIRMAN OF THE BOARD

        The chairman of the Board, if such an officer were elected, shall, if present, preside at meetings of the Board of Directors and exercise and perform such other powers and duties as may from time to time be assigned to him by the Board of Directors or as may be prescribed by these bylaws. If there is no president, then the chairman of the Board shall also be the chief executive officer of the Corporation and shall have the powers and duties prescribed in Section 5.7 of these bylaws.

5.7   PRESIDENT

        Subject to such supervisory powers, if any, as may be given by the Board of Directors to the chairman of the Board, if there be such an officer, the president shall be the chief executive officer of the Corporation and shall; subject to the control of the Board of Directors, have general supervision, direction, and control of the business and the officers of the Corporation. He shall preside at all meetings of the stockholders and, in the absence or nonexistence of a chairman of the Board, at all meetings of the Board of Directors. He shall have the general powers and duties of management usually vested in the office of president of a Corporation and shall have such other powers and duties as may be prescribed by the Board of Directors or these bylaws.

5.8   VICE PRESIDENT

        In the absence or disability of the president, the vice presidents, if any, in order of their rank as fixed by the Board of Directors or, if not ranked, a vice president designated by the Board of Directors, shall perform all the duties of the president and when so acting shall have all the powers of, and be subject to all the restrictions upon, the president. The vice presidents shall have such other powers and perform such other duties as from time to time may be prescribed for them respectively by the Board of Directors, these bylaws, the president or the chairman of the Board.

5.9   SECRETARY

        The secretary shall keep or cause to be kept, at the principal executive office of the Corporation or such other place as the Board of Directors may direct, a book of minutes of all meetings and actions of directors, committees of directors, and stockholders. The minutes shall show the time and place of each meeting, whether regular or special (and, if special, how authorized and the notice given), the names of those present at directors’ meetings or committee meetings, the number of shares present or represented at stockholders’ meetings, and the proceedings thereof.

        The secretary shall keep, or cause to be kept, at the principal executive office of the Corporation or at the office of the Corporation’s transfer agent or registrar, as determined by resolution of the Board of Directors, a stock ledger, or a duplicate stock ledger, showing the names of all stockholders and their addresses, the number and classes of shares held by each, the number and date of certificates evidencing such shares, and the number and date of cancellation of every certificate surrendered for cancellation.

        The secretary shall give, or cause to be given, notice of all meetings of the stockholders and of the Board of Directors required to be given by law or by these bylaws. He shall keep the seal of the Corporation, if one be adopted, in safe custody and shall have such other powers and perform such other duties as may be prescribed by the Board of Directors or by these bylaws.

5.10  TREASURER

        The treasurer shall keep and maintain, or cause to be kept and maintained, adequate and correct books and records of accounts of the properties and business transactions of the Corporation, including accounts of its assets, liabilities, receipts, disbursements, gains, losses, capital, retained earnings, and shares. The books of account shall at all reasonable times be open to inspection by any director.

        The treasurer shall deposit all money and other valuables in the name and to the credit of the Corporation with such depositaries as may be designated by the Board of Directors. He shall disburse the funds of the Corporation as may be ordered by the Board of Directors, shall render to the president and directors, whenever they request it, an account of all of his transactions as treasurer and of the financial condition of the Corporation, and shall have such other powers and perform such other duties as may be prescribed by the Board of Directors or these bylaws:

5.11  ASSISTANT SECRETARY

        The assistant secretary, or, if there is more than one, the assistant secretaries in the order determined by the stockholders or Board of Directors (or if there be no such determination, then in the order of their election) shall, in the absence of the secretary or in the event of his or her inability or refusal to act, perform the duties and exercise the powers of the secretary and shall perform such other duties and have such other powers as the Board of Directors or the stockholders may from time to time prescribe.

5.12  ASSISTANT TREASURER

        The assistant treasurer, or, if there is more than one, the assistant treasurers, in the order determined by the stockholders or Board of Directors (or if there be no such determination, then in the order of their election), shall, in the absence of the treasurer or in the event of his or her inability or refusal to act, perform the duties and exercise the powers of the treasurer and shall perform such other duties and have such other powers as the Board of Directors or the stockholders may from time to time prescribe.

5.13  AUTHORITY AND DUTIES OF OFFICERS

        In addition to the foregoing authority and duties, all officers of the Corporation shall respectively have such authority and perform such duties in the management of the business of the Corporation as may be designated from time to time by the Board of Directors or the stockholders.

ARTICLE VI

INDEMNITY

6.1   INDEMNIFICATION OF DIRECTORS AND OFFICERS

        The Corporation shall, to the maximum extent and in the manner permitted by the General Corporation Law of Delaware, indemnify each of its directors and officers against expenses (including attorneys’ fees), judgments, fines, settlements, and other amounts actually and reasonably incurred in connection with any proceeding, arising by reason of the fact that such person is or was an agent of the Corporation. For purposes of this Section 6.1, a “director” or “officer” of the Corporation includes any person (i) who is or was a director or officer of the Corporation, (ii) who is or was serving at the request of the Corporation as a director or officer of another corporation, partnership, joint venture, trust or other enterprise, or (iii) who was a director or officer of a corporation which was a predecessor corporation of the Corporation or of another enterprise at the request of such predecessor corporation.

6.2   INDEMNIFICATION OF OTHERS

        The Corporation shall have the power, to the extent and in the manner permitted by the General Corporation Law of Delaware, to indemnify each of its employees and agents (other than directors and officers) against expenses (including attorneys’ fees), judgments, fines, settlements, and other amounts actually and reasonably incurred in connection with any proceeding, arising by reason of the fact that such person is or was an agent of the Corporation. For purposes of this Section 6.2, an “employee” or “agent” of the Corporation (other than a director or officer) includes any person (i) who is or was an employee or agent of the Corporation, (ii) who is or was serving at the request of the Corporation as an employee or agent of another corporation, partnership, joint venture, trust or other enterprise, or (iii) who was an employee or agent of a corporation which was a predecessor corporation of the Corporation or of another enterprise at the request of such predecessor corporation.

6.3   INSURANCE

        The Corporation may purchase and maintain insurance on behalf of any person who is or was a director, officer, employee or agent of the Corporation, or is or was serving at the request of the Corporation as a director, officer, employee or agent of another corporation, partnership, joint venture, trust or other enterprise against any liability asserted against him and incurred by him in any such capacity, or arising out of his status as such, whether or not the Corporation would have the power to indemnify him against such liability under the provisions of the General Corporation Law of Delaware.

ARTICLE VII

RECORDS AND REPORTS

7.1   MAINTENANCE AND INSPECTION OF RECORDS

        The Corporation shall, either at its principal executive office or at such place or places as designated by the Board of Directors, keep a record of its stockholders listing their names and addresses and the number and class of shares held by each stockholder, a copy of these bylaws as amended to date, accounting books, and other records.

        Any stockholder of record, in person or by attorney or other agent, shall, upon written demand under oath stating the purpose thereof, have the right during the usual hours for business to inspect for any proper purpose the Corporation’s stock ledger, a list of its stockholders, and its other books and records and to make copies or extracts therefrom. A proper purpose shall mean a purpose reasonably related to such person’s interest as a stockholder. In every instance where an attorney or other agent is the person who seeks the right to inspection, the demand under oath shall be accompanied by a power of attorney or such other writing that authorizes the attorney or other agent to so act on behalf of the stockholder. The demand under oath shall be directed to the Corporation at its registered office in Delaware or at its principal place of business.

        The officer who has charge of the stock ledger of a Corporation shall prepare and make, at least ten (10) days before every meeting of stockholders, a complete list of the stockholders entitled to vote at the meeting, arranged in alphabetical order, and showing the address of each stockholder and the number of shares registered in the name of each stockholder. Such list shall be open to the examination of any stockholder, for any purpose germane to the meeting, during ordinary business hours, for a period of at least ten (10) days prior to the meeting, either at a place within the city where the meeting is to be held, which place shall be specified in the notice of the meeting, or, if not so specified, at the place where the meeting is to be held. The list shall also be produced and kept at the time and place of the meeting during the whole time thereof, and may be inspected by any stockholder who is present.

7.2   INSPECTION BY DIRECTORS

        Any director shall have the right to examine the Corporation’s stock ledger, a list of its stockholders, and its other books and records for a purpose reasonably related to his position as a director. The Court of Chancery is hereby vested with the exclusive jurisdiction to determine whether a director is entitled to the inspection sought. The Court may summarily order the Corporation to permit the director to inspect any and all books and records, the stock ledger, and the stock list and to make copies or extracts therefrom. The Court may, in its discretion, prescribe any limitations or conditions with reference to the inspection, or award such other and further relief as the Court may deem just and proper.

7.3   ANNUAL STATEMENT TO STOCKHOLDERS

        The Board of Directors shall present at each annual meeting, and at any special meeting of the stockholders when called for by vote of the stockholders, a full and clear statement of the business and condition of the Corporation.

7.4   REPRESENTATION OF SHARES OF OTHER CORPORATIONS

        The chairman of the Board, the president, any vice president, the treasurer, the secretary or assistant secretary of this Corporation, or any other person authorized by the Board of Directors or the president or a vice president, is authorized to vote, represent, and exercise on behalf of this Corporation all rights incident to any and all shares of any other corporation or corporations standing in the name of this Corporation. The authority granted herein may be exercised either by such person directly or by any other person authorized to do so by proxy or power of attorney duly executed by such person having the authority.

ARTICLE VIII

GENERAL MATTERS

8.1   CHECK

        From time to time, the Board of Directors shall determine by resolution which person or persons may sign or endorse all checks, drafts, other orders for payment of money, notes or other evidences of indebtedness that are issued in the name of or payable to the Corporation, and only the persons so authorized shall sign or endorse those instruments.

8.2   EXECUTION OF CORPORATE CONTRACTS AND INSTRUMENTS

        The Board of Directors, except as otherwise provided in these bylaws, may authorize any officer or officers, or agent or agents, to enter into any contract or execute any instrument in the name of and on behalf of the Corporation; such authority may be general or confined to specific instances. Unless so authorized or ratified by the Board of Directors or within the agency power of an officer, no officer, agent or employee shall have any power or authority to bind the Corporation by any contract or engagement or to pledge its credit or to render it liable for any purpose or for any amount.

8.3   STOCK CERTIFICATES; PARTLY PAID SHARES

        The shares of a Corporation shall be represented by certificates, provided that the Board of Directors of the Corporation may provide by resolution or resolutions that some or all of any or all classes or series of its stock shall be uncertificated shares. Any such resolution shall not apply to shares represented by a certificate until such certificate is surrendered to the Corporation. Notwithstanding the adoption of such a resolution by the Board of Directors, every holder of stock represented by certificates and upon request every holder of uncertificated shares shall be entitled to have a certificate signed by, or in the name of the Corporation by the chairman or vice-chairman of the Board of Directors, or the president or vice president, and by the treasurer or an assistant treasurer, or the secretary or an assistant secretary of such Corporation representing the number of shares registered in certificate form. Any or all of the signatures on the certificate may be a facsimile. In case any officer, transfer agent or registrar who has signed or whose facsimile signature has been placed upon a certificate has ceased to be such officer, transfer agent or registrar before such certificate is issued, it may be issued by the Corporation with the same effect as if he were such officer, transfer agent or registrar at the date of issue.

        The Corporation may issue the whole or any part of its shares as partly paid and subject to call for the remainder of the consideration to be paid therefor. Upon the face or back of each stock certificate issued to represent any such partly paid shares, upon the books and records of the Corporation in the case of uncertificated partly paid shares, the total amount of the consideration to be paid therefor and the amount paid thereon shall be stated. Upon the declaration of any dividend on fully paid shares, the Corporation shall declare a dividend upon partly paid shares of the same class, but only upon the basis of the percentage of the consideration actually paid thereon.

8.4   SPECIAL DESIGNATION ON CERTIFICATES

        If the Corporation is authorized to issue more than one class of stock or more than one series of any class, then the powers, the designations, the preferences, and the relative, participating, optional or other special rights of each class of stock or series thereof and the qualifications, limitations or restrictions of such preferences and/or rights shall be set forth in full or summarized on the face or back of the certificate that the Corporation shall issue to represent such class or series of stock; provided, however, that, except as otherwise provided in Section 202 of the General Corporation Law of Delaware, in lieu of the foregoing requirements there may be set forth on the face or back of the certificate that the Corporation shall issue to represent such class or series of stock a statement that the Corporation will furnish without charge to each stockholder who so requests the powers, the designations, the preferences, and the relative participating, optional or other, special rights of each class of stock or series thereof and the qualifications, limitations or restrictions of such preferences and/or rights.

8.5   LOST CERTIFICATES

        Except as provided in this Section 8.5, no new certificates for shares shall be issued to replace a previously issued certificate unless the latter is surrendered to the Corporation and canceled at the same time. The Corporation may issue a new certificate of stock or uncertificated shares in the place of any certificate theretofore issued by it, alleged to have been lost, stolen or destroyed, and the Corporation may require the owner of the lost, stolen or destroyed certificate, or his legal representative, to give the Corporation a bond sufficient to indemnify it against any claim that may be made against it on account of the alleged loss, theft or destruction of any such certificate or the issuance of such new certificate or uncertificated shares.

8.6   CONSTRUCTIONS DEFINITIONS

        Unless the context requires otherwise, the general provisions, rules of construction, and definitions in the Delaware General Corporation Law shall govern the construction of these bylaws. Without limiting the generality of this provision, the singular number includes the plural, the plural number includes the singular, and the term “person” includes both a corporation and a natural person.

8.7   DIVIDENDS

        The directors of the Corporation, subject to any restrictions contained in the certificate of incorporation, may declare and pay dividends upon the shares of its capital stock pursuant to the General Corporation Law of Delaware. Dividends may be paid in cash, in property, or in shares of the Corporation’s capital stock.

        The directors of the Corporation may set apart out of any of the funds of the Corporation available for dividends a reserve or reserves for any proper purpose and may abolish any such reserve. Such purposes shall include but not be limited to equalizing dividends, repairing or maintaining any property of the Corporation, and meeting contingencies.

8.8   FISCAL YEAR

        The fiscal year of the Corporation shall be fixed by resolution of the Board of Directors and may be changed by the Board of Directors.

        The Corporation shall adopt a corporate seal, which may be altered at pleasure, and use the same by causing it or a facsimile thereof, to be impressed or affixed or in any other manner reproduced.

8.9   TRANSFER OF STOCK

        Upon surrender to the Corporation or the transfer agent of the Corporation of a certificate for shares duly endorsed or accompanied by proper evidence of succession, assignation or authority to transfer, it shall be the duty of the Corporation to issue a new certificate to the person entitled thereto, cancel the old certificate, and record the transaction in its books.

8.10  STOCK TRANSFER AGREEMENTS

        The Corporation shall have power to enter into and perform any agreement with any number of stockholders of any one or more classes of stock of the Corporation to restrict the transfer of shares of stock of the Corporation of any one or more classes owned by such stockholders in any manner not prohibited by the General Corporation Law of Delaware.

8.11  REGISTERED STOCKHOLDERS

        The Corporation shall be entitled to recognize the exclusive right of a person registered on its books as the owner of shares to receive dividends and to vote as such owner, shall be entitled to hold liable for calls and assessments the person registered on its books as the owner of shares, and shall not be bound to recognize any equitable or other claim to or interest in such share or shares on the part of another person, whether or not it shall have express or other notice thereof, except as otherwise provided by the laws of Delaware.

ARTICLE IX

AMENDMENTS

        The original or other bylaws of the Corporation may be adopted, amended or repealed by the stockholders entitled to vote; provided, however, that the Corporation may, in its certificate of incorporation, confer the power to adopt, amend or repeal bylaws upon the directors. The fact that such power has been so conferred upon the directors shall not divest the stockholders of the power, nor limit their power to adopt, amend or repeal bylaws.

ARTICLE X

DISSOLUTION

        If it should be deemed advisable in the judgment of the Board of Directors of the Corporation that the Corporation should be dissolved, the Board, after the adoption of a resolution to that effect by a majority of the whole Board at any meeting called for that purpose, shall cause notice to be mailed to each stockholder entitled to vote thereon of the adoption of the resolution and of a meeting of stockholders to take action upon the resolution.

        At the meeting a vote shall be taken for and against the proposed dissolution. If a majority of the outstanding stock of the Corporation entitled to vote thereon votes for the proposed dissolution, then a certificate stating that the dissolution has been authorized in accordance with the provisions of Section 275 of the General Corporation Law of Delaware and setting forth the names and residences of the directors and officers shall be executed, acknowledged, and filed and shall become effective in accordance with Section 103 of the General Corporation Law of Delaware. Upon such certificate’s becoming effective in accordance with Section 103 of the General Corporation Law of Delaware, the Corporation shall be dissolved.

        Whenever all the stockholders entitled to vote on a dissolution consent in writing, either in person or by duly authorized attorney, to a dissolution, no meeting of directors or stockholders shall be necessary. The consent shall be filed and shall become effective in accordance with Section 103 of the General Corporation Law of Delaware. Upon such consent’s becoming effective in accordance with Section 103 of the General Corporation Law of Delaware, the Corporation shall be dissolved. If the consent is signed by an attorney, then the original power of attorney or a photocopy thereof shall be attached to and filed with the consent. The consent filed with the Secretary of State shall have attached to it the affidavit of the secretary or some other officer of the Corporation stating that the consent has been signed by or on behalf of all the stockholders entitled to vote on a dissolution; in addition, there shall be attached to the consent a certification by the secretary or some other officer of the Corporation setting forth the names and residences of the directors and officers of the Corporation.

ARTICLE XI

CUSTODIAN

11.1  APPOINTMENT OF A CUSTODIAN IN CERTAIN CASES

        The Court of Chancery, upon application of any stockholder, may appoint one or more persons to be custodians and, if the Corporation is insolvent, to be receivers, of and for the Corporation when:

(i)    at any meeting held for the election of directors the stockholders are so divided that they have failed to elect successors to directors whose terms have expired or would have expired upon qualification of their successors; or

(ii)    the business of the Corporation is suffering or is threatened with irreparable injury because the directors are so divided respecting the management of the affairs of the Corporation that the required vote for action by the Board of Directors cannot be obtained and the stockholders are unable to terminate this division; or

(iii) the Corporation has abandoned its business and has failed within a reasonable time to take steps to dissolve, liquidate or distribute its assets.

11.2  DUTIES OF CUSTODIAN

        The custodian shall have all the powers and title of a receiver appointed under Section 291 of the General Corporation Law of Delaware, but the authority of the custodian shall be to continue the business of the Corporation and not to liquidate its affairs and distribute its assets, except when the Court of Chancery otherwise orders and except in cases arising under Sections 226(a)(3) or 352(a)(2) of the General Corporation Law of Delaware.

CERTIFICATE OF SECRETARY I, the undersigned, do hereby certify: 1.   That I am the duly elected and acting Secretary of BioMarin Pharmaceutical Inc.; and

        2. _______ That the foregoing Bylaws, comprising 21 pages, constitute the Bylaws of said Corporation as duly adopted by the Board of Directors of the Corporation on April 2, 2001.

        IN WITNESS WHEREOF, I have hereunto subscribed my name and affixed the seal of the Corporation as of the ___ day of _______, 2001.

- -------------------------------------------------------------------------------------------------
Raymond W. Anderson, Secretary

Table of Contents                                                           Page
- -----------------                                                           ----

ARTICLE I             CORPORATE OFFICES........................................1
         1.1          REGISTERED OFFICE........................................1
         1.2          OTHER OFFICES............................................1

ARTICLE II            MEETINGS OF STOCKHOLDERS.................................1
         2.1          PLACE OF MEETINGS........................................1
         2.2          ANNUAL MEETING...........................................1
         2.3          SPECIAL MEETING..........................................1
         2.4          NOTICE OF STOCKHOLDERS' MEETINGS.........................2
         2.5          MANNER OF GIVING NOTICE: AFFIDAVIT OF NOTICE.............2
         2.6          QUORUM...................................................2
         2.7          ADJOURNED MEETING; NOTICE................................2
         2.8          VOTING...................................................2
         2.9          WAIVER OF NOTICE.........................................3
         2.10         STOCKHOLDER ACTION BY WRITTEN CONSENT WITHOUT A MEETING..3
         2.11         RECORD DATE FOR STOCKHOLDER NOTICE; VOTING
                      GIVING CONSENTS..........................................4
         2.12         PROXIES..................................................4
         2.13         LIST OF STOCKHOLDERS ENTITLED TO VOTE....................5

ARTICLE III           DIRECTORS................................................5
         3.1          POWERS...................................................5
         3.2          NUMBER OF DIRECTORS......................................5
         3.3          ELECTION QUALIFICATION AND TERM OF OFFICE OF DIRECTORS...6
         3.4          RESIGNATION AND VACANCIES................................6
         3.5          PLACE OF MEETINGS; MEETINGS BY TELEPHONE.................7
         3.6          FIRST MEETINGS...........................................7
         3.7          REGULAR MEETINGS.........................................7
         3.8          SPECIAL MEETINGS; NOTICE.................................7
         3.9          QUORUM...................................................8
         3.10         WAIVER OF NOTICE.........................................8
         3.11         ADJOURNED MEETING: NOTICE................................8
         3.12         BOARD ACTION BY WRITTEN CONSENT WITHOUT A MEETING........9
         3.13         FEES AND COMPENSATION OF DIRECTORS.......................9
         3.14         APPROVAL OF LOANS TO OFFICERS............................9
         3.15         REMOVAL OF DIRECTORS.....................................9

ARTICLE IV            COMMITTEES...............................................9
         4.1          COMMITTEES OF DIRECTORS..................................9
         4.2          COMMITTEE MINUTES.......................................10
         4.3          MEETINGS AND ACTION OF COMMITTEES.......................10

ARTICLE V             OFFICERS................................................11
         5.1          OFFICERS................................................11
         5.2          ELECTION OF OFFICERS....................................11
         5.3          SUBORDINATE OFFICERS....................................11
         5.4          REMOVAL AND RESIGNATION OF OFFICERS.....................11
         5.5          VACANCIES IN OFFICES....................................12
         5.6          CHAIRMAN OF THE BOARD...................................12
         5.7          PRESIDENT...............................................12
         5.8          VICE PRESIDENT..........................................12
         5.9          SECRETARY...............................................12
         5.10         TREASURER...............................................13
         5.11         ASSISTANT SECRETARY.....................................13
         5.12         ASSISTANT TREASURER.....................................13
         5.13         AUTHORITY AND DUTIES OF OFFICERS........................14

ARTICLE VI            INDEMNITY...............................................14
         6.1          INDEMNIFICATION OF DIRECTORS AND OFFICERS...............14
         6.2          INDEMNIFICATION OF OTHERS...............................14
         6.3          INSURANCE...............................................15

ARTICLE VII           RECORDS AND REPORTS.....................................15
         7.1          MAINTENANCE AND INSPECTION OF RECORDS...................15
         7.2          INSPECTION BY DIRECTORS.................................16
         7.3          ANNUAL STATEMENT TO STOCKHOLDERS........................16
         7.4          REPRESENTATION OF SHARES OF OTHER CORPORATIONS..........16

ARTICLE VIII          GENERAL MATTERS.........................................16
         8.1          CHECK...................................................16
         8.2          EXECUTION OF CORPORATE CONTRACTS AND INSTRUMENTS........17
         8.3          STOCK CERTIFICATES; PARTLY PAID SHARES..................17
         8.4          SPECIAL DESIGNATION ON CERTIFICATES.....................17
         8.5          LOST CERTIFICATES.......................................18
         8.6          CONSTRUCTIONS DEFINITIONS...............................18
         8.7          DIVIDENDS...............................................18
         8.8          FISCAL YEAR.............................................19
         8.9          TRANSFER OF STOCK.......................................19
         8.10         STOCK TRANSFER AGREEMENTS...............................19
         8.11         REGISTERED STOCKHOLDERS.................................19

ARTICLE IX            AMENDMENTS..............................................19

ARTICLE X             DISSOLUTION.............................................20

ARTICLE XI            CUSTODIAN...............................................20
         11.1         APPOINTMENT OF A CUSTODIAN IN CERTAIN CASES.............20
         11.2         DUTIES OF CUSTODIAN.....................................21

EX-10

LICENSE AGREEMENT

        This License Agreement is made and entered into by and between BioMarin Pharmaceutical Inc., having its principal place of business at 371 Bel Marin Keys Boulevard, Suite 210, Novato, CA 94949 ("BioMarin") and W. R. Grace & Co., having its principal place of business at 7500 Grace Drive, Columbia, MD 21046 ("Grace"). Each of BioMarin and Grace may be referred to herein individually as a "Party" and collectively as the "Parties."

        WHEREAS, Grace owns certain Patent Rights and Related Technology, as defined below; and

        WHEREAS, BioMarin was previously granted an Option to acquire an exclusive worldwide license thereunder, in the Option Agreement dated May 1, 1998 between the Parties (“Option Agreement”); and

        WHEREAS, BioMarin has now exercised its Option for such exclusive license;

        NOW, THEREFORE, in consideration of the premises and mutual covenants contained herein, BioMarin and Grace hereby agree as follows:

ARTICLE I - DEFINITIONS

        1.01   “Affiliate” means any corporation or other business entity which directly or indirectly controls, is controlled by, or is under common control with one of the Parties hereto. “Controls” (including “controlled by” and “under common control with”) means the possession, direct or indirect, of the power to direct or cause the direction of the management and policies of a corporation or other business entity, whether through the ownership of voting securities, by contract or otherwise.

        1.02  "Agreement" means this License Agreement.

        1.03   “ANS” or “Annual Net Sales” shall mean the total of the Net Sales Prices for all Licensed Products sold by BioMarin, BioMarin sublicensees and BioMarin distributors to third parties during a Contract Year.

        1.04  "BioMarin" shall mean BioMarin Pharmaceutical Inc., having a place of business at 371 Bel Marin Keys Boulevard, Suite 210, Novato, CA 94949.

        1.05   “Contract Year” shall mean each successive twelve (12) month period during the term of this Agreement beginning on the Effective Date or anniversaries thereof.

        1.06   “Earned Royalty” shall mean the royalties based on sales or transfers of Licensed Product to third parties, as specified in Paragraph 3.03.

        1.07  "Effective Date" shall mean January 1, 2001.

        1.08   “First Commercial Sale” shall mean the first sale for commercial purposes by BioMarin or a BioMarin sublicensee or a BioMarin distributor of a Licensed Product following approval by the Federal Food & Drug Administration (if in the United States) or other appropriate regulatory agency (if outside the United States). For the purpose of this definition, “commercial purposes” is not intended to mean or include sales or transfers made solely for research purposes or for clinical trial purposes (even if token remuneration is received) or for inventory purposes (consignment inventory or inventory sold to a distributor at below market prices but not yet sold to a third party).

        1.09  "Grace" shall mean W. R. Grace & Co., having its principal place of business at 7500 Grace Drive, Columbia, MD 21044, and Affiliates thereof.

        1.10  "License Maintenance Fee" shall mean the annual fee payable by BioMarin to Grace pursuant to Paragraph 3.01 for each Contract Year up to and including the Contract Year in which the First Commercial Sale is made.

        1.11   “Licensed Product” shall mean any product whose manufacture, use, offer for sale or sale would infringe a Valid Claim in the country for which such product is manufactured, used, offered for sale or sold.

        1.12   “Minimum Annual Royalty” shall mean the minimum royalty payable in each Contract Year following the First Commercial Sale of Licensed Product, as specified by Paragraph 3.02, in order for BioMarin to maintain its license in the event that Earned Royalties for that Contract Year are zero or less than the minimum.

        1.13   “Net Sales Price” means the sales price for Licensed Product sold by BioMarin or a BioMarin sublicensee or a BioMarin distributor to third parties, less (i) sales, excise, or use taxes separately stated on the face of the invoice, (ii) transportation or insurance charges separately stated and itemized on the face of the invoice, (iii) customs duties, and (iv) all return, trade and discount allowances or credits actually given.

        1.14  "Patent Rights" shall mean all subject matter claimed in the following:

        (a)   United States Patent Numbers 5,145,681 and 5,505,943 and any continuations, continuations-in-part, divisions and substitutions of any such patent and all renewals, reissues and extensions of any such patent; and

        (b)   Any foreign patents and/or applications that are counterparts of a patent described in subparagraph 1.14(a), including any patent or application filed by or on behalf of Grace that claims subject matter claimed in, or takes priority from, a patent described in Subparagraph 1.14(a). Such foreign patents and/or applications as of the Effective Date are listed in Appendix A, attached hereto and made a part of this Agreement.

        1.15   “Related Technology” shall mean any biological materials, and any research and development information, inventions, know-how, and all pre-clinical, clinical and other technical data, in each case, that were owned by Grace, or possessed by Grace with the right to provide to others, as of May 1, 1998, the effective date of the Option Agreement, which in each case relate to the practice of the Patent Rights. The Related Technology has already been provided to BioMarin.

        1.16   “Valid Claim” shall mean a claim of an issued patent included within the Patent Rights, which has not been held unenforceable or invalid by a court or other governmental agency of competent jurisdiction, and which has not been admitted to be invalid or unenforceable through reissue, disclaimer or otherwise.

ARTICLE II - LICENSE GRANT

        2.01   Subject to payment of the fees and royalties specified in Article III below, Grace hereby grants to BioMarin an exclusive, worldwide, royalty-bearing license under the Patent Rights and Related Technology, to make, use, offer to sell and sell Licensed Products, to practice any process, method or procedure of the Patent Rights and Related Technology and to otherwise exploit the Patent Rights and Related Technology.

        2.02   BioMarin’s license rights include the right to grant sublicenses to practice under the Patent Rights and Related Technology within the scope of BioMarin’s license rights, subject to the following:

        (a)  Any sublicense granted by BioMarin shall contain the following provisions:

          i.  confidentiality obligations as in Article IV;

          ii.  record and audit provisions as in Paragraph 2.04;

          iii.  an agreement by the sublicensee to cooperate with Grace and BioMarin in any actions pursuant to Paragraph 7.02 to prevent or eliminate infringement of any Patent Rights;

          iv.  compliance provisions as in Paragraphs 5.04, 5.05, and 10.04;

          v.  termination provisions as in Article VIII; and

          vi.  acknowledgment by the sublicensee that it is not acquiring any right, title or interest in or to the Patent Rights and Related Technology or any portion thereof, except as expressly permitted by Grace’s license to BioMarin in this Agreement, and that upon termination of this Agreement all rights thereby acquired by each sublicensee shall immediately and automatically extinguish.

        (b)   Practice of the Patent Rights and Related Technology by such BioMarin sublicensee(s) shall be subject to Earned Royalties as if practiced by BioMarin, and BioMarin shall pay Earned Royalties to Grace on behalf of its sublicensee(s) pursuant to Paragraphs 3.03 through 3.06.

         (c)  BioMarin shall promptly notify Grace of each sublicense granted.

         (d)  BioMarin and Grace shall share equally in any upfront or other fees paid by BioMarin's sublicensee(s) in respect of the sublicense(s); provided, however, that earned or running royalties paid by BioMarin’s sublicensee(s) based on sales of Licensed Product to third parties shall not be subject to the sharing provisions of this subparagraph.

        2.03   Related Technology used or useful in connection with BioMarin’s activities pursuant to this Agreement has previously been provided to BioMarin by Grace. Grace has previously supplied BioMarin, at no additional cost to BioMarin, with viable cultures of microorganisms capable of producing Vibriolysin. Grace has previously provided BioMarin, at no additional cost to BioMarin, with copies of all Grace patents within the Patent Rights. Grace is required to provide any additional Related Technology (as detailed in the Option Agreement) that might become known to Grace, but shall have no obligation to conduct any research with respect to the subject matter of the Patent Rights and Related Technology.

        2.04   BioMarin shall keep books of record sufficient to verify BioMarin’s compliance with the license provisions of this Article and to verify the accuracy and completeness of the accounting under Article III, and shall cause all its sublicensees to do the same. Such books of record shall be preserved for a period not less than six years after their creation. BioMarin agrees to take all reasonably necessary steps to make these books of record available for inspection and copying by Grace, or Grace’s designated representative, at Grace’s sole expense, upon reasonable notice and during normal business hours but not more than once during any twelve (12) month period during the term of this Agreement.

ARTICLE III - - LICENSE MAINTENANCE FEES & ROYALTIES

        3.01   License Maintenance Fees - As partial consideration for the license rights granted herein, BioMarin shall pay an annual License Maintenance Fee of XXXX. The first License Maintenance Fee shall be due ten (10) days following the date of signature of the last Party to execute this Agreement. Subsequent annual License Maintenance Fee payments shall be due on the anniversaries of the Effective Date.

        3.02   Minimum Annual Royalty - A Minimum Annual Royalty payment of XXXX. The Minimum Annual Royalty paid for each Contract Year shall be applied as a credit against Earned Royalties payable in that Contract Year. There shall be no refund of any Minimum Annual Royalty and there shall be no carryover of Minimum Annual Royalty to any subsequent Contract Year.

        3.03   Earned Royalty - Subject to application of the royalty credit of Paragraph 3.02, an Earned Royalty shall be payable by BioMarin to Grace based on the cumulative Annual Net Sales (“ANS”) of Licensed Products for each Contract Year, based on the following rate schedule:

        Annual Net Sales ("ANS")                Royalty

        Through XXXXXXX                XXX of ANS

        Over XXXXXXX,                XXX of ANS XXXX
        through XXXXXXX                through XXXXX

        Over XXXXX                XXX of ANS over XXXXX

For example, if the cumulative ANS for a Contract Year is XXXXXX, the total Earned Royalty payable to Grace for that Contract Year would be:

        XXXXXX + XXXXXX + XXXXXX = XXXXXX.

        3.04  Earned Royalties shall be calculated and payable XXXXXXX. Subject to application of the royalty credit for Minimum Annual Royalty payments pursuant to Paragraph 3.02, payment of the amount of the calculated Earned Royalties shall be paid to Grace within sixty (60) days following the end of each calendar quarter. Notwithstanding the foregoing, in the event of any intellectual property claim, cause of action or litigation asserted against BioMarin as a result of its practice of the Patent Rights or Related Technology, BioMarin shall have the right to withhold XXXXXX of the Earned Royalties otherwise payable hereunder and to apply such withheld amounts against the costs and expenses incurred by BioMarin in connection with such claim, cause of action or litigation, until such time as the claim, cause of action or litigation has been fully and finally resolved, following which full payment of Earned Royalties would resume.

        3.05  A full accounting of the calculation of the Earned Royalties by BioMarin, including the country in which each Licensed Product was sold by or on behalf of BioMarin or its sublicensee(s), shall accompany each royalty payment, and such an accounting shall be delivered to Grace even if no Earned Royalties are payable for that quarter.

        3.06  All royalties due Grace by BioMarin under this Agreement (either minimum annual or earned royalties) shall be payable in U.S. dollars, with the rate of exchange for any non-U.S. currencies received for any sales being the exchange rate specified in the American edition of the Wall Street Journal. U.S.-dollar equivalence shall be calculated on a quarterly basis, using the average of the exchange rate on the first and last business day of the quarter for each of the currencies in which sales of Licensed Product(s) were made during the quarter.

ARTICLE IV - CONFIDENTIALITY

        4.01  During the Term of this Agreement and for a period of two (2) years thereafter, BioMarin and Grace each shall use their best efforts to protect the confidentiality of proprietary information provided to it by the other Party and identified in writing or orally as confidential and proprietary and shall not use such information except for the purposes of this Agreement. This obligation of confidentiality shall not apply to information which:

          (a)  is or becomes known publicly through no fault of the receiving Party;

          (b)  is obtained by the receiving Party from a third party entitled to disclose it;

          (c)  is already known to the receiving Party at the time of disclosure under this Agreement or under previous agreements between the Parties, including the Option Agreement; or

          (d)  is developed by the receiving Party independent of any disclosure made hereunder or under previous agreements between the Parties, including the Option Agreement, which can be demonstrated by written or other documentary evidence.

        4.02  The confidentiality obligations of this Article IV do not apply when such disclosure of information is required by law. Nothing in the confidentiality obligations of this Article IV shall be construed to prevent BioMarin from any sale or use of any Licensed Product.

ARTICLE V - REPRESENTATIONS, WARRANTIES, AND INDEMNIFICATION

        5.01  Grace represents and warrants that:

          (a)  Grace owns all rights, title and interest in and to the Patent Rights and Related Technology;

          (b)  As of the date of the Option Agreement (May 1, 1998), Grace was not aware of any patent or other intellectual property rights of third parties which would be infringed by practice of the Patent Rights or Related Technology;

          (c)  Grace has the right to enter into this Agreement, to fully comply with the terms and conditions of this Agreement and to grant to BioMarin the rights and licenses granted herein; and

          (d)  Grace has not executed any agreement or contract that is inconsistent with the rights and licenses granted hereunder.

          5.02  BioMarin represents and warrants that:

          (a)  BioMarin has the right to enter into this Agreement and to fully comply with the terms and conditions of this Agreement;

          (b)  BioMarin shall use its best efforts to commence clinical testing of Licensed Products after the Effective Date of this Agreement; and

          (c) BioMarin shall use its best efforts to obtain regulatory clearance for and to commercialize Licensed Product.

        5.03  Nothing in this Agreement shall be read or construed as:

          (a)  a warranty or representation as to the commercial utility of the Licensed Product or any product based on the Patent Rights or Related Technology;

          (b)  a warranty or representation that any pending application within the Patent Rights will mature into an issued patent;

          (c)   a warranty or representation as to the validity or scope of any Patent Rights; or

          (d)  a warranty or representation that anything made, used, or otherwise disposed of under this Agreement is or will be free from infringement of patents, copyrights or trademarks of third parties; or

          (e)  an obligation on the part of Grace to bring or prosecute actions or suits against third parties for infringement.

        5.04  As between Grace and BioMarin, BioMarin shall have sole control and responsibility for the manufacture, use and/or sale of Licensed Products. BioMarin shall bear the full responsibility for any liability to any customers and others for any Licensed Product made by or on behalf of BioMarin or any BioMarin sublicensee or any BioMarin distributor, and shall indemnify and hold Grace and its corporate Affiliates harmless from any judgment, claim, expense or liability arising out of the sale of Licensed Product(s) by or on behalf of BioMarin and/or any BioMarin sublicensee and/or any BioMarin distributor (including direct, consequential and punitive damages).

        5.05  BioMarin further warrants that any manufacture, use and/or sale of Licensed Product(s) by or on behalf of BioMarin or any BioMarin sublicensee shall be in substantial compliance with all material laws, regulations, and necessary governmental permits and approvals that are applicable.

ARTICLE VI - PATENT PROSECUTION & MAINTENANCE

        6.01  Grace shall use reasonable efforts to prosecute, as Grace sees fit, the pending patent applications within the Patent Rights, using counsel selected by Grace and with due consultation with BioMarin. Grace shall provide BioMarin with copies of all relevant documentation so that BioMarin may be informed and apprised of the continuing prosecution; BioMarin agrees to keep this documentation confidential. Grace shall use reasonable efforts, with due consideration to the stage of prosecution, to amend any patent application within the Patent Rights to include claims requested by BioMarin to protect the products contemplated by BioMarin to be sold hereunder.

        6.02  Grace agrees that no patent or pending patent application within the Patent Rights will be allowed to lapse or go abandoned without first offering such case(s) to BioMarin.

        6.03  BioMarin shall be responsible for all out-of-pocket costs and expenses in connection with the prosecution and maintenance of the Patent Rights following the Effective Date. Grace shall submit to BioMarin itemized invoices for such costs on a quarterly basis and BioMarin shall reimburse Grace for such expenses within sixty (60) days. BioMarin will have the right to request and receive from Grace an annual budget for patent prosecution and maintenance. BioMarin will have the right to review and negotiate this budget within the standard of reasonable pharmaceutical industry practice. This annual budget will not be exceeded by greater than five percent (5%) without prior consultation with and agreement of BioMarin.

ARTICLE VII - PATENT ENFORCEMENT AND THIRD PARTY PATENTS

        7.01  Grace intends to protect its interests in any Grace patents from infringement by third parties and to prosecute infringers or otherwise act to eliminate infringement when, in the sole judgement of Grace, such action is necessary, proper and justified.

        7.02  In the event BioMarin believes there is substantial infringement of any patent in the Patent Rights, which alleged infringement is to the material detriment of BioMarin, BioMarin shall notify Grace and supply such evidence as it has of the alleged infringement. Grace shall have the sole right and discretion with respect to preventing and/or causing the discontinuance of any infringement of Grace patents. BioMarin agrees that it will, at Grace's request and expense, furnish such reasonable assistance as may be required to assist in preventing or discontinuing any such infringement. In the event that Grace fails or refuses, within one hundred twenty (120) days after notice of any alleged infringement of the Patent Rights, to initiate proceedings to prevent and/or to take action to cause the discontinuance of such alleged infringement, BioMarin upon notice to Grace may at its option undertake any such legal proceedings at its own cost and expense, and BioMarin shall be solely entitled to all recoveries from such litigation. In any legal proceedings brought by BioMarin pursuant to this Paragraph, BioMarin agrees to keep Grace advised and informed, and Grace may be represented by counsel of its choice, at Grace's sole expense, acting in an advisory capacity. BioMarin may not enter into any settlement agreement adversely affecting the validity or enforceability of any of the Patent Rights without the express prior written approval of Grace.

ARTICLE VIII - TERM AND TERMINATION

        8.01  This Agreement shall become effective as of the Effective Date. Unless earlier terminated, the Agreement shall remain in full force and effect until the last to expire of the patents within the Patent Rights.

        8.02  Grace shall have the right to terminate this Agreement only in the event of material breach or default by BioMarin. It is understood and agreed that any breach or default in connection with the obligation to pay fees or royalties hereunder shall be deemed material. Termination under this Paragraph shall be automatically effective if such breach or default has not been cured (a) within thirty (30) calendar days after written notice of the breach to BioMarin if the claimed breach is a failure to pay any amount due and owing hereunder, or (b) within ninety (90) calendar days after written notice of the breach to BioMarin if the claimed breach is any other material default hereunder. Termination under this Paragraph shall automatically terminate any and all sublicenses granted hereunder by BioMarin.

        8.03  BioMarin shall have the right to terminate its license rights under this Agreement for any or no reason (i.e., arbitrarily), effective upon ninety (90) days notice in writing to Grace. BioMarin may so terminate with respect to the entire Agreement or with respect to one or more patent applications within the Patent Rights; provided, however, that in the latter case, there shall be no change in the royalty obligations under Article III. Termination of the entire Agreement under this Paragraph shall automatically terminate any and all sublicenses granted hereunder by BioMarin. Termination with respect to one or more patent applications within the Patent Rights shall automatically result in a similar termination of any and all sublicenses granted hereunder by BioMarin with respect to such patent application(s).

        8.04  For any quantity of Licensed Product which BioMarin or a BioMarin sublicensee has in its possession or is in the process of manufacturing at the time termination is effective under Paragraph 8.02 or 8.03, BioMarin may elect to have that quantity of the Licensed Product be subject to the terms of the license as if the Agreement had not been terminated and may sell or transfer such Licensed Product to third parties for a period of up to six (6) months, or may elect to have it destroyed or the manufacture thereof canceled.

        8.05  At Grace's option, BioMarin shall, upon termination of this Agreement, either return to Grace all confidential information received pursuant to this Agreement or the Option Agreement, or certify that such information has been destroyed. At BioMarin's option, Grace shall, upon termination of this Agreement, either return to BioMarin all confidential information received pursuant to this agreement or the Option Agreement, or certify that such information has been destroyed. Notwithstanding the preceding, each Party would be permitted to retain one copy of the confidential information, solely for record purposes.

        8.06  Expiration or termination of this Agreement shall not discharge, affect or modify any rights or obligations which accrue or are incurred prior to the date of expiration or termination. For example, there shall be no refund or credit of any fees or royalties paid or accrued prior to the date expiration or termination is effective.

        8.07  Sections 2.04, 3.03 (to the extent provided in Paragraph 8.04), 3.04, 3.05, 3.06, and 4.2, and Articles I, IV, and VIII to X shall survive any expiration or termination of this Agreement.

ARTICLE IX - ASSIGNABILITY

        9.01  Neither this Agreement nor the rights and obligations of either Party hereunder shall be assigned without the prior written consent of the other Party; provided, however, that such consent will not be unreasonably withheld for companies within the biopharmaceutical or healthcare industries that are equal to or exceed the commercial capabilities and financial resources of BioMarin at the time of execution of this Agreement. Notwithstanding the foregoing, either Party may freely assign this Agreement to any affiliate or to a successor of the entire business to which this Agreement relates.

        9.02  All of the terms, covenants, representations, warranties and conditions of this Agreement shall be binding upon, and inure to the benefit of and be enforceable by, the Parties hereto and their respective successors.

ARTICLE X - MISCELLANEOUS

        10.01  This Agreement shall be construed and interpreted, and the rights and obligations of the Parties shall be determined, in accordance with the laws of the State of California, without regard to the conflicts of laws principles thereof.

        10.02  Dispute resolution - In the event of any controversy or claim arising out of or relating to any provisions of this Agreement or the breach thereof, the Parties shall try to settle those conflicts amicably between themselves. Should they fail to agree, the matter in dispute shall be settled through arbitration conducted by and in accordance with the rules of the American Arbitration Association ("AAA"), except as modified by this Paragraph 10.02. Either Party may furnish the other Party with a dated, written notice (the "Arbitration Notice") indicating (i) intent to commence arbitration proceedings, (ii) the nature, with reasonable detail, of the dispute, and (iii) the remedy sought. Each arbitration shall be conducted by three arbitrators experienced in pharmaceutical licensing, consisting of one arbitrator chosen by each Party and the third arbitrator chosen by the first two. In the event that the first two arbitrators are not able to agree upon and chose a third arbitrator, the third arbitrator shall be appointed in accordance with the AAA rules. The arbitration decision shall be binding and not appealable to any court in any jurisdiction. The prevailing Party may enter the arbitration decision in any court having competent jurisdiction.

        10.03  No alteration or amendment of this Agreement shall be effective unless embodied in writing and executed by an authorized representative of each Party.

        10.04  Export Laws - BioMarin agrees to abide by any restrictions or conditions respecting the export or re-export of Grace information disclosed in connection with this Agreement which is subject to export control under the Export Administration Regulations of the United States Department of Commerce, or export control regulations of other United States Government agencies including the Department of State and Department of Treasury. BioMarin agrees that it will not export such information, or the direct product thereof, directly or indirectly, to any countries to which such export is now or hereafter becomes illegal under any such regulations. Any breach of the terms of this paragraph by BioMarin shall constitute a material breach, entitling Grace to terminate pursuant to Paragraph 8.02. BioMarin further agrees to require all BioMarin sublicensees to abide by the provisions of this Paragraph.

        10.05  Notices - All notices required to be given under this Agreement, and the payment of required fees and royalties, shall be made by first class air mail or express courier addressed to the respective Party at the address below, or by telefax to the numbers listed below (for notices), or to such changed address or telefax number as a Party may designate in writing to the other Party. Such notice, if received, shall be deemed to have been received, if by first class mail, on the fifteen (15th) calendar day after posting; if by express courier, on the date of delivery by the courier; and if by telefax, upon transmission with electronic confirmation of receipt.

        If to Grace:    Attn:   Dr. Nils Friis
                        W.R. Grace & Co.
                        5400 Broken Sound Boulevard NW, Suite 300
                        Boca Raton FL  33487-2702

                        Telefax #:  (561) 362-1323

        with a copy to: Attn:  Chief Patent Counsel
                        W.R. Grace & Co.
                        7500 Grace Drive
                        Columbia MD  21044

                        Telefax #:  (410) 531-4195

        If to BioMarin: Attn:  William Anderson, Cheif Operating and Financial Officer
                        BioMarin Pharmaceutical Inc., Suite 210
                        371 Bel Marin Keys Boulevard, Suite 210
                        Novato, CA  94949

                        Telefax #:  (415) 382-7889

        10.06  Force Majeure - Each Party shall be excused from the performance of its obligations hereunder in the event that such performance is prevented by force majeure, provided that each Party so affected shall use commercially reasonable efforts to alleviate the force majeure condition or to complete such performance by other means. For the purpose of this Agreement, force majeure is defined as an event beyond the control of the Parties and independent from their will, including (but not limited to) strikes or other labor trouble, war, insurrection, fire, flood, explosion, discontinuity in the supply of power or other critical components, court order or governmental interference and acts of God.

        10.07  The Parties to this Agreement are independent contractors and not joint venturers or partners, and no agency, partnership, or power to obligate the other Party is created by this Agreement.

        10.08  If any of the provisions of this Agreement are or become in conflict with the laws or regulations of any jurisdiction or any governmental entity having jurisdiction over the Parties or the subject matter, those provisions shall be deleted and the remaining terms and conditions of this Agreement shall remain in full force and effect.

        10.09  This Agreement constitutes the entire understanding of the Parties with respect to the subject matter hereof and supersedes and previous understandings and agreements, written or oral, which between the Parties hereto may have reached with respect to the subject matter, including the Option Agreement.

IN WITNESS WHEREOF, the Parties hereto have duly executed this Agreement in duplicate originals.

BIOMARIN PHARMACEUTICAL INC.

By:    /s/ Raymond W. Anderson
       ------------------------------------
Name:  Raymond W. Anderson
Title: Chief Financial and Operating Officer
Date:  March 28, 2001


W. R. GRACE & CO.

By:    /s/ Kevin Coghlan
       -------------------------------------
Name:  Kevin Coghlan
Title: Vice President, Business Development
Date: March 26, 2001

Attachments: Appendix A - Patent Rights

10-Q

United States
Securities and Exchange Commission
Washington, D.C. 20549
FORM 10-Q

 (Mark One)
 [X]     QUARTERLY REPORT UNDER SECTION 13 OR 15(D) OF THE SECURITIES EXCHANGE ACT OF 1934

                  For the quarterly period ended March 31, 2001


[  ]     TRANSITION REPORT PURSUANT TO SECTION 13 OR 15(D) OF THE SECURITIES

         EXCHANGE ACT OF 1934

                  For the transition period from ______ to _________.


                      Commission file number: 000-26727

BIOMARIN PHARMACEUTICAL INC.

          (Exact name of registrant issuer as specified in its charter)

           Delaware                                  68-0397820
(State or other jurisdiction of            (I.R.S. Employer Identification No.)
incorporation or organization)

          371 Bel Marin Keys Blvd., Suite 210, Novato, California 94949
                    (address of principal executive offices)
                                   (Zip Code)
                                 (415) 884-6700
              (Registrant's telephone number, including area code)

(Former name, former address and former fiscal year, if changed since last report)

     Indicate  by check mark  whether the  registrant  (1) has filed all reports
required to be filed by Section 13 or 15(d) of the  Securities  Exchange  Act of
1934 during the past 12 months (or for such shorter  period that the  registrant
was  required  to file such  reports),  and (2) has been  subject to such filing
requirements for the past 90 days. Yes _X___ No_____

                APPLICABLE ONLY TO ISSUERS INVOLVED IN BANKRUPTCY
                   PROCEEDINGS DURING THE PRECEDING FIVE YEARS

     Indicate  by check mark  whether the  registrant  filed all  documents  and
reports  required  to be filed by  Sections  12,  13 or 15(d) of the  Securities
Exchange Act of 1934 subsequent to the  distribution of securities  under a plan
confirmed by a court. Yes ____ No_____

                      APPLICABLE ONLY TO CORPORATE ISSUERS


     Indicate the number of shares  outstanding of each of the issuer's  classes
of common stock, as of the latest  practicable  date:  37,155,257  shares common
stock, par value $0.001, outstanding as of May 1, 2001.

BIOMARIN PHARMACEUTICAL INC.

TABLE OF CONTENTS

                                                                            Page
PART I.  FINANCIAL INFORMATION                                              ----

Item 1.  Financial Statements (Unaudited).

Consolidated Balance Sheets....................................................2
Consolidated Statements of Operations for the three-month
    periods ended March 31, 2000 and 2001 and for the period
    from March 21, 1997 (inception) through March 31, 2001.....................3
Consolidated Statements of Cash Flows..........................................4
Notes to Consolidated Financial  Statements....................................5

Item 2.  Management's Discussion and Analysis..................................7

Item 3.   Quantitative and Qualitative Disclosure about Market Risk...........17


PART II. OTHER INFORMATION

Item 1.  Legal Proceedings....................................................18

Item 2.  Changes in Securities and Uses of Proceeds...........................18

Item 3.  Defaults upon Senior Securities......................................18

Item 4.  Submission of Matters to a Vote of Security Holders..................18

Item 5.  Other Information....................................................18

Item 6.  Exhibits and Reports on Form 8-K.....................................18


SIGNATURE.....................................................................20

Item 1.  Financial Statements (Unaudited)
                  BioMarin Pharmaceutical Inc. and Subsidiaries
                          (a development-stage company)

                        Consolidated Balance Sheets as of
                      December 31, 2000 and March 31, 2001
                                 (In Thousands)

                                                  December 31,        March 31,
                                                     2000              2001
                                              -----------------   --------------
Assets                                                              (unaudited)
Current assets:
 Cash and cash equivalents                          $   16,530         $ 19,722
 Short-term investments                                 23,671           10,044
 Accounts receivable, net                                1,135              754
 Due from BioMarin/Genzyme LLC                           1,799            3,386
 Inventories                                               436              439
 Prepaid expenses                                          970            1,516
                                              -----------------   --------------
  Total current assets                                  44,541           35,861

Property, plant and equipment, net                      20,715           19,983
Goodwill and other intangible assets                     9,862            9,333
Investment in BioMarin/Genzyme LLC                       1,482             (217)
Deposits                                                   333              483
                                              -----------------   --------------
  Total assets                                      $   76,933         $ 65,443
                                              =================   ==============

Liabilities and Stockholders' Equity
Current liabilities:
 Accounts payable                                   $    4,747         $  1,616
 Accrued liabilities                                     2,109            1,992
 Notes payable, short-term                                  27               29
                                              -----------------   --------------
  Total current liabilities                              6,883            3,637

 Long-term portion of notes payable                         56               47
                                              -----------------   --------------
  Total liabilities                                      6,939            3,684
                                              -----------------   --------------
Stockholders' equity:
 Common stock, $0.001 par value:  75,000,000
 authorized.   36,921,966 and 37,134,859 shares
 issued and outstanding December 31, 2000
 and March 31, 2001, respectively.                          37               37
 Additional paid-in capital                            153,940          155,220
 Deferred compensation                                  (1,530)          (1,321)
 Notes from stockholders                                (1,940)          (1,964)
 Deficit accumulated during development stage          (80,513)         (90,213)
                                              -----------------   --------------
  Total stockholders' equity                            69,994           61,759
                                              -----------------   --------------
  Total liabilities and stockholders' equity        $   76,933         $ 65,443
                                              =================   ==============

        The accompanying notes are an integral part of these statements.
                                        2

                  BioMarin Pharmaceutical Inc. and Subsidiaries
                          (a development-stage company)

                      Consolidated Statements of Operations
        For the Three-Month Periods Ended March 31, 2000 and 2001 and for
        the Period from March 21, 1997 (inception) through March 31, 2001
              (In Thousands, except for per share data, Unaudited)

                                                                  Period from
                                                                 March 21, 1997
                                        Three Months Ended       (Inception) to
                                             March 31,              March 31,
                                     ------------------------
                                          2000         2001             2001
                                     -------------------------------------------
Revenues:
 Revenues - products                   $   469       $   692        $     4,576
 Revenues - services                        50            17                464
 Revenues from BioMarin/Genzyme LLC      2,756         2,691             18,559
 Revenues - other                           23             -                293
                                     -------------------------------------------
  Total revenues                         3,298         3,400             23,892
                                     -------------------------------------------
Operating Costs and Expenses:
 Cost of products                          148           266              1,312
 Cost of services                           33             5                250
 Research and development                8,663         9,984             85,400
 Selling, general and administrative     1,996         2,203             22,268
 Carson Street closure                   4,423             -              4,423
                                     -------------------------------------------
  Total operating costs and expenses    15,263        12,458            113,653
                                     -------------------------------------------
Loss from operations                   (11,965)       (9,058)           (89,761)

Interest income                            788           468              6,029
Interest expense                            (2)           (2)              (741)
Loss from BioMarin/Genzyme LLC            (557)       (1,108)            (5,740)
                                     -------------------------------------------
Net loss                             $ (11,736)     $ (9,700)       $   (90,213)
                                     ===========================================
Net loss per share,
  basic and diluted                   $  (0.34)     $  (0.26)       $     (3.50)
                                     ===========================================
Weighted average common shares
 outstanding, basic and diluted         35,015        37,052             25,791
                                     ===========================================

        The accompanying notes are an integral part of these statements.
                                       3

                  BioMarin Pharmaceutical Inc. and Subsidiaries
                          (a development-stage company)

                      Consolidated Statements of Cash Flows
       For the Three-Month Periods Ended March 31, 2000 and 2001, and for
          the Period from March 21, 1997 (inception) to March 31, 2001
                            (In Thousands, Unaudited)
                                                                    Period from
                                                                     March 21,
                                                  Three Months          1997
                                                      Ended          (inception)
                                                     March 31,           to
                                              --------------------    March 31,
                                                  2000        2001      2001
                                              ----------------------------------
Cash flows from operating activities:
 Net loss                                       $(11,736)  $ (9,700)   $(90,213)
 Adjustments to reconcile net
   loss to net cash used in
   operating activities:
  Depreciation                                     1,163      1,162       9,896
  Amortization of deferred compensation              268        209       3,121
  Amortization of goodwill                           303        529       3,543
  Loss from BioMarin/Genzyme LLC                   3,304      3,799      23,454
  Compensation in the form of common stock
     and common stock options                          -          -          18
  Write off of in-process technology                   -          -       2,625
  Carson Street closure                            3,791          -       3,791

Changes in operating assets and liabilities:
  Accounts receivable, net                           (86)       381        (754)
  Due from BioMarin/Genzyme LLC                   (2,453)    (1,587)     (3,386)
  Inventories                                         29         (3)        161
  Prepaid expenses                                    69       (546)     (1,516)
  Deposits                                           (95)      (150)       (483)
  Accounts payable                                (1,728)    (3,131)      1,616
  Accrued liabilities                              1,328       (117)      1,992
                                              ----------------------------------
 Total adjustments                                 5,893        546      44,078
                                              ----------------------------------
  Net cash used in operating activities           (5,843)    (9,154)    (46,135)
                                              ----------------------------------
Cash flows from investing activities:
 Purchase of property and equipment                 (608)      (430)    (33,669)
 Investment in BioMarin/Genzyme LLC               (3,674)    (2,100)    (23,237)
 Sale (purchase) of short-term investments        24,574     13,627     (10,044)
 Purchase of Biochemical Research Reagent
   Division of Oxford Glycosciences, Plc.              -          -      (1,500)
                                              ----------------------------------
  Net cash provided by (used in)
   investing activities                           20,292     11,097     (68,450)
                                              ----------------------------------
Cash flows from financing activities:
 Proceeds from exercise of common
   stock options and warrants                      2,821        408       7,033
 Repayment of equipment loan                         (8)         (7)        (59)
 Proceeds from sale of common stock,
   net of issuance costs                              -         848      99,774
 Proceeds from note payable                           -           -         134
 Proceeds from issuance of
   convertible notes payable                          -           -      25,615
  Repayment of notes from stockholders                -           -         804
 Issuance of common stock for ESPP                    -           -         314
                                                      -           -         692
                                               ---------------------------------
  Net cash provided by financing activities       2,813       1,249     134,307
                                               ---------------------------------
Net increase in cash and cash equivalents        17,262       3,192      19,722
Cash and cash equivalents,
  beginning of period                            23,413      16,530           -
                                               ---------------------------------
Cash and cash equivalents, end of period        $40,675     $19,722    $ 19,722
                                               =================================
        The accompanying notes are an integral part of these statements.
                                        4
                                    

BIOMARIN PHARMACEUTICAL INC. AND SUBSIDIARIES

(a development-stage company)

NOTES TO CONSOLIDATED FINANCIAL STATEMENTS

(Unaudited)

1.    BASIS OF PRESENTATION:

BioMarin Pharmaceutical Inc. (the Company) is a biopharmaceutical company specializing in the development of enzyme therapies for debilitating life-threatening chronic genetic disorders and other diseases and conditions. Since inception, the Company has devoted substantially all of its efforts to research and development activities, including preclinical studies and clinical trials, the establishment of laboratory, clinical and commercial scale manufacturing facilities, clinical manufacturing, and related administrative activities.

The Company was incorporated on October 25, 1996 in the state of Delaware and first began business on March 21, 1997 (inception) as a wholly-owned subsidiary of Glyko Biomedical Ltd. (GBL). Subsequently, the Company has issued stock to outside investors in a series of transactions, resulting in GBL’s ownership of the Company’s outstanding common stock being reduced to 30.6 percent at March 31, 2001.

On October 7, 1998, the Company acquired Glyko, Inc., a wholly-owned subsidiary of GBL, in a transaction valued at $14.5 million. The transaction was accounted for as a purchase and resulted in Glyko, Inc. becoming a wholly-owned subsidiary of the Company. Glyko, Inc. provides products and services that perform sophisticated carbohydrate analysis for research institutions and commercial laboratories.

In January 2001, BioMarin signed an agreement with Acqua Wellington North American Equities Fund Ltd. (Acqua Wellington) for an equity investment in the Company. Under the terms of the agreement, the Company will have the option to request Acqua Wellington to invest through sales of registered common stock at a small discount to market price. The maximum amount that the Company may request to be invested in any one month is dependent upon the market price of the stock (or can be mutually agreed-upon by both parties) and is referred to as the "Draw Down Amount." Acqua Wellington is obligated to purchase this amount if requested to do so by the Company. In addition, the Company, at its discretion, may grant a "Call Option" to Acqua Wellington for an additional investment in an amount up to the "Draw Down Amount" which Acqua Wellington may or may not choose to exercise. In the initial transaction under this agreement on February 2, 2001, Acqua Wellington purchased 101,523 shares for $1 million ($848,000 net of issuance costs, the majority of which will be nonrecurring upon future transactions associated with this agreement). As of May 4, 2001, the potential maximum future amount available to the Company under the agreement is $49 million.

Through March 31, 2001, the Company had accumulated losses during its development stage of approximately $90.2 million. Based on current plans, management expects to incur further losses at least through 2002. Management believes that the Company’s cash and cash equivalents and short-term investment balances at March 31, 2001 will be sufficient to meet the Company’s obligations at least through 2001. Until we can generate sufficient levels of cash from our operations, we expect to continue to finance future cash needs through the sale of equity securities, equipment-based financing, and collaborative agreements with corporate partners. Management is currently pursing various alternatives to support its operations beyond 2001.

The accompanying unaudited consolidated financial statements have been prepared in accordance with generally accepted accounting principles for interim financial information on substantially the same basis as the annual audited financial statements. However, they do not include all of the information and footnotes required by generally accepted accounting principles for complete financial statements. In the opinion of management, all adjustments, consisting of normal recurring adjustments, considered necessary for a fair presentation have been included. Operating results for the three-month period ended March 31, 2001 are not necessarily indicative of the results that may be expected for the year ending December 31, 2001. These consolidated financial statements should be read in conjunction with the financial statements and footnotes thereto for the year ended December 31, 2000 included in the Company’s Form 10-K Annual Report.

2.    SIGNIFICANT ACCOUNTING POLICIES:

Use of Estimates

The preparation of financial statements in conformity with generally accepted accounting principles requires management to make certain estimates and assumptions that affect the reported amounts of assets and liabilities, disclosure of contingent assets and liabilities at the dates of the financial statements, and the reported amounts of revenues and expenses during the reporting period. Actual results could differ from those estimates. Significant estimates made by management include determination of progress to date of research and development projects in-process, the amortization period of goodwill and other intangibles, and asset impairment reserves related to certain leasehold improvements and equipment.

                                        5

Cash and Cash Equivalents

For the consolidated statements of cash flows, the Company treats liquid investments with original maturities of less than three months when purchased as cash and cash equivalents.

Short-term Investments

The Company records its investments as held-to-maturity. These investments are recorded at cost at March 31, 2001, which approximates fair market value. These securities are comprised mainly of Federal Agency investments, including Federal Home Loans and Federal Farm Credits.

Investment in BioMarin/Genzyme LLC and Related Revenue

Under the terms of the Company’s joint venture agreement with Genzyme, the Company and Genzyme have each agreed to provide 50 percent of the funding for the joint venture. All research and development, sales and marketing, and other activities performed by Genzyme and the Company on behalf of the joint venture are billed to the joint venture at cost. Any profits or losses of the joint venture are shared equally by the two parties. Losses of the joint venture are allocated in proportion to the funding provided by each joint venture partner.

The Company accounts for its investment in the joint venture using the equity method. The percentage of the research and development costs incurred by the Company and billed to the joint venture that was funded by the Company was recorded as a credit to the Company’s equity in the loss of the joint venture.

Property and Equipment

Property and equipment are stated at cost. Depreciation is computed using the straight-line method. Leasehold improvements are amortized over the life of the asset or the term of the lease, whichever is shorter. Significant additions and improvements are capitalized, while repairs and maintenance are charged to expense as incurred.

Property and equipment consisted of the following (in thousands):

                                       December 31,    March 31,    Estimated
                                         2000            2001      Useful Lives
                                      ---------        -------- ----------------
Computer hardware and software         $   678         $   795      3 years
Office furniture and equipment           1,056           1,180      5 years
Manufacturing/laboratory equipment       9,323           9,644      5 years
Leasehold improvements                  16,685          16,546  Shorter of life
                                                                   of asset or
                                                                   lease term
Construction in progress                 1,048           1,055
                                      ---------        --------
                                        28,790          29,220
Less:  Accumulated depreciation         (8,075)         (9,237)
                                      ---------        --------
                Total, net             $20,715         $19,983
                                      =========        ========

Research and Development

Research and development expenses include the expenses associated with contract research and development provided by third parties, research and development provided in connection with the BioMarin/Genzyme LLC joint venture including clinical manufacturing, clinical operations and regulatory costs, and internal research and development costs. All research and development costs discussed above are expensed as incurred.

                                        6

Net Income (Loss) per Share

Basic net income (loss) per share is calculated by dividing net income (loss) by the weighted average common shares outstanding during the period. Diluted net income per share is calculated by dividing net income by the weighted average of common stock outstanding and potential common shares during the period. Potential common shares include dilutive shares issuable upon the exercise of outstanding common stock options, warrants, and contingent issuances of common stock. For periods in which the Company has losses, such potential common shares are excluded from the computation of diluted net loss per share, as their effect is anti-dilutive.

3.    CARSON STREET CLOSURE:

During the first quarter of 2000, the Company decided to close its Carson Street clinical manufacturing facility. In connection with this decision the Company recorded a charge of approximately $4.4 million. The charge primarily consisted of impairment reserves for leasehold improvements and equipment located in the Carson Street facility.

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Item 2.     Management's Discussion and Analysis of Financial Condition and Results of Operations

FORWARD-LOOKING STATEMENTS

The following “Management’s Discussion and Analysis of Financial Condition and Results of Operations” contains“forward-looking statements” as defined under securities laws. These statements can often be identified by the use of terminology such as “believes,” “expects,” “anticipates,” “plans,” “may,” “will,” “projects,” “continues,” “estimates,” “potential,” “opportunity” and so on. These forward-looking statements may be found in the “Factors that May Affect Future Results,” and other sections of this document. Our actual results or experience could differ significantly from the forward-looking statements. Factors that could cause or contribute to these differences include those discussed in “Factors that May Affect Future Results,” as well as those discussed elsewhere in this document.

You should not place undue reliance on these statements, which speak only as of the date that they were made. These cautionary statements should be considered in connection with any written or oral forward-looking statements that we may issue in the future. We do not undertake any obligation to release publicly any revisions to these forward-looking statements after completion of the filing of this Form 10-Q to reflect later events or circumstances or to reflect the occurrence of unanticipated events.

Overview

We are a developer of enzyme therapies for debilitating, life-threatening, chronic genetic diseases and other diseases or conditions. Since our inception on March 21, 1997, we have been engaged in research and development activities, including preclinical studies, clinical trials and clinical manufacturing, the establishment of laboratory and manufacturing facilities, and administrative activities.

We have incurred net losses since inception and had an accumulated deficit through March 31, 2001 of $90.2 million. Our losses have resulted primarily from research and development activities and related administrative expenses. We expect to continue to incur operating losses at least through 2002.

To date, we have not generated revenues from the sale of our drug candidates. Our lead product is Aldurazyme, laronidase for injection, (recombinant human (alpha)-L-iduronidase), which is undergoing clinical trials for use in enzyme replacement therapy for Mucopolysaccharidosis-I or MPS-I. We have initiated a clinical trial of rhASB (recombinant human N-acetylgalactosamine-4 sulfatase), for use in enzyme replacement therapy for the treatment of MPS-VI or Maroteaux-Lamy Syndrome. We have also successfully conducted preclinical studies in pigs and mice of our burn debridement (cleaning) enzyme, Vibriolysin, for use in wound debridement in preparation for skin grafting and expect to submit an application to the U.S. Food and Drug Administration (FDA) or foreign equivalent to begin a clinical trial by mid-2001.

In January 2001, we signed an agreement with Acqua Wellington North American Equities Fund Ltd. (Acqua Wellington) for an equity investment in the our company. Under the terms of the agreement, we will have the option to request Acqua Wellington to invest through sales of our registered common stock at a small discount to market price. The maximum amount that we may request to be invested in any one month is dependent upon the market price of our stock (or can be mutually agreed-upon by both parties) and is referred to as the "Draw Down Amount." Acqua Wellington is obligated to purchase this amount if requested to do so by us. In addition we, at our discretion, may grant a "Call Option" to Acqua Wellington for an additional investment in an amount up to the "Draw Down Amount" which Acqua Wellington may or may not choose to exercise. In the initial transaction under this agreement on February 2, 2001, Acqua Wellington purchased 101,523 shares of our common stock for $1 million ($848,000 net of issuance costs, the majority of which will be nonrecurring upon future transactions associated with this agreement). As of May 4, 2001, the potential maximum future amount available to us under the agreement is $49 million.

Results of Operations

The Quarters Ended March 31, 2001 and 2000

Revenues for the first quarter of 2001 totaled $3.4 million compared to revenues of $3.3 million in the first quarter of 2000. First quarter 2001 revenues included $2.7 million for services provided to the joint venture for Aldurazyme compared to $2.8 million in the same period in 2000. The decrease was primarily the result of efficiencies gained in the manufacturing of Aldurazyme. First quarter 2001 revenues also included $709,000 generated by Glyko, Inc. compared to $519,000 for the first quarter of 2000 primarily as a result of increased sales in both the United States and European sales offices.

                                        8

Cost of products and cost of services related to Glyko, Inc. operations were $271,000 in the first quarter of 2001 and were $181,000 in the comparable period of 2000. Glyko’s total external product and service costs as a percent of the sales of products and services were 38% in the first quarter of 2001 and 35% in the first quarter of 2000. The change was due primarily to a less favorable revenue mix, with a greater percentage of lower margin product sales.

Research and development expenses for the first quarter of 2001 increased by $1.3 million to $10.0 million from $8.7 million in the first quarter of 2000. Increased expenses in support of the rhASB and Vibriolysin programs were the major factors in the growth of research and development expenses.

Selling, general and administrative expenses increased to $2.2 million in the first quarter of 2001 from $2.0 million in the first quarter 2000. This increase was primarily due to the acceleration of the amortization of goodwill for the purchase of Glyko, Inc. The estimated life of the goodwill was decreased from ten years to seven years in the third quarter of 2000.

In the first quarter of 2000, we recorded a provision of $4.4 million for the closure of our Carson Street clinical manufacturing facility. The provision primarily consisted of impairment reserves for leasehold improvements and equipment located in the Carson Street facility.

The equity in the loss of our joint venture with Genzyme was $1.1 million for the first quarter 2001 compared to $557,000 for the same period of 2000 as the joint venture continued its original clinical trial and began its Phase III clinical trial of Aldurazyme late in 2000.

Interest income was $468,000 for the first quarter of 2001 compared to $788,000 for the same period of 2000. The decrease was primarily due to decreased interest rates and the reduction of cash, cash equivalents and short-term investment balances available for investment in 2001 .

Net loss was $9.7 million ($0.26 per share, basic and diluted) in the first quarter of 2001 compared to a net loss of $11.7 million ($0.34 per share, basic and diluted) in the comparable period of 2000.

Liquidity and Capital Resources

We have financed our operations since our inception by the issuance of common stock and convertible notes and the related interest income earned on cash balances available for short-term investment. Since inception, we have raised aggregate net proceeds of approximately $134.3 million. We were initially funded by GBL with a $1.5 million investment. We have since raised additional capital from the sale of common stock in private placements, the sale of promissory notes convertible into common stock, an investment of $8.0 million by Genzyme as part of our joint venture with them, an initial public offering including the underwriters’ over-allotment exercise, the concurrent $10.0 million Genzyme investment in our Company, the sale of $1.0 million of common stock to Acqua Wellington, and sales from stock option and warrant exercises and the Employee Stock Purchase Plan.

Our combined cash, cash equivalents and short-term investments totaled $29.8 million at March 31, 2001, a decrease of $10.4 million for the quarter. The primary uses of cash during the three months ended March 31, 2001 were to finance operations, fund the joint venture and purchase equipment and leasehold improvements. The primary source of cash during this period was the sale of $1.0 million of common stock to Acqua Wellington and the issuance of common stock pursuant to the exercise of stock options under the 1997 Stock Option Plan. For the three months ended March 31, 2001, operations used $9.2 million, we invested $2.1 million in the joint venture (which was consumed in joint venture operations), we purchased $430,000 of equipment and leasehold improvements, we raised $408,000 from the exercise of stock options and we received $848,000 (net of issuance costs) from the sale of common stock to Acqua Wellington.

From our inception through March 31, 2001, we have purchased approximately $33.7 million of leasehold improvements and equipment. We expect that our investment in leasehold improvements and equipment will increase significantly during the next two years because we will provide facilities and equipment for a larger staff and increase manufacturing capacity for both commercial and clinical requirements.

As part of the acquisition of Glyko, Inc., we acquired in-process research and development projects, the value of which was expensed as a portion of the purchase price at the time of the acquisition. The 11 projects acquired are each relatively small and can be grouped into two categories, analytic projects and diagnostic projects.

                                        9

The analytic projects are intended to expand the analytic product line by adding new enzymes for reagent sales, new kits for agricultural applications, new instrument capabilities for protein analysis and a major upgrade of software capabilities. At the time of the acquisition of Glyko, Inc., all of the analytic projects had completed feasibility work and the software projects were 75% complete and have since been completed. The development of specialized materials supporting instrument capabilities is deemed to be the most difficult technical hurdle for the completion and commercialization of the analytic projects. The fair value of the analytic projects was $1.7 million at the time of the acquisition.

The diagnostic projects are intended to expand a product line based on very precise measurements of the level of complex carbohydrates in blood and urine as indicators of serious disease conditions including heart disease, kidney disease and mucopolysaccharidoses or carbohydrate lysosomal storage diseases. At the time of the Glyko, Inc. acquisition, preliminary feasibility work had been done for all of the projects and a software project was well advanced as to programming, which has since been completed. The development of new more sensitive carbohydrate chemistry techniques is deemed to be the most difficult technical hurdle for the completion and commercialization of the diagnostic products. The fair value of the diagnostic projects was $924,000 at the time of the acquisition.

As of March 31, 2001, we had expended to date approximately $1.1 million on the analytic projects and $1.2 million on the diagnostic projects. If all acquired in-process research and development projects proceed to completion, we expect to spend approximately $100,000 in incremental direct expense to complete the analytic projects in phases over approximately 3 months. We expect to spend approximately $300,000 to complete the diagnostic projects in phases within the next 6 months. None of these projects have been terminated to date.

Since the acquisition of these in-process research and development projects, there have been no subsequent developments which indicate that the completion and commercialization of either of the projects are less likely to be completed on the original planned schedule or less likely to be a commercial success.

We have made and plan to make substantial commitments to capital projects, including expanding the Aldurazyme and rhASB manufacturing facilities, developing new research and development facilities, and expanding our administrative and support offices.

In September 1998, we established a joint venture with Genzyme for the worldwide development and commercialization of Aldurazyme for the treatment of MPS-I. We share expenses and profits from the joint venture equally with Genzyme. Genzyme purchased $8.0 million in common stock upon signing the agreement and $10.0 million of common stock at the IPO price of $13 per share in a private placement concurrent with the initial public offering. Genzyme has committed to pay us an additional $12.1 million upon approval by the FDA of the biologics license application (BLA) for Aldurazyme as a treatment for MPS-I.

In January 2001, we signed an agreement with Acqua Wellington North American Equities Fund Ltd. (Acqua Wellington) for an equity investment in the our company. Under the terms of the agreement, we will have the option to request Acqua Wellington to invest through sales of our registered common stock at a small discount to market price. The maximum amount that we may request to be invested in any one month is dependent upon the market price of our stock (or can be mutually agreed-upon by both parties) and is referred to as the "Draw Down Amount." Acqua Wellington is obligated to purchase this amount if requested to do so by us. In addition we, at our discretion, may grant a "Call Option" to Acqua Wellington for an additional investment in an amount up to the "Draw Down Amount" which Acqua Wellington may or may not choose to exercise. In the initial transaction under this agreement on February 2, 2001, Acqua Wellington purchased 101,523 shares of our common stock for $1 million ($848,000 net of issuance costs, the majority of which will be nonrecurring upon future transactions associated with this agreement). As of May 4, 2001, the potential maximum future amount available to us under the agreement is $49 million.

The net proceeds from any sales of our common stock to Acqua Wellington will be used to fund operating costs, capital expenditures and working capital requirements, which may include costs associated with our lead clinical programs including Aldurazyme for MPS-I, rhASB for MPS-VI and our program for Vibriolysin, which is being studied for the debridement (cleaning) of severe burns. In addition, net proceeds may also be used for the research and development of other pipeline products, building of the Company’s supporting infrastructure, and other general corporate purposes.

We expect our current funds to last at least through 2001. Until we can generate sufficient levels of cash from our operations, we expect to continue to finance future cash needs through:

    .  The sale of equity securities

    .  Equipment-based financing

    .  Collaborative agreements with corporate partners

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We do not expect to generate positive cash flow from operations at least through 2002 because we expect to increase operational expenses and manufacturing investment for the joint venture and to increase research and development activities, including:

    .  Preclinical studies, clinical trials and regulatory review

    .  Development of manufacturing operations

    .  Process development activities

    .  Scale-up of manufacturing processes in larger capacity facilities

We anticipate a need for additional financing to fund the future operations of our business, including the commercialization of our drug candidates currently under development. We are actively considering our financing options to support our operations beyond 2001. We cannot assure you that additional financing will be obtained or, if obtained, will be available on reasonable terms or in a timely manner.

Our future capital requirements will depend on many factors, including, but not limited to:

    .  The progress of our research and development programs

    .  The progress of preclinical studies and clinical trials

    .  The time and cost involved in obtaining regulatory approvals

    .  Scaling up, installing and validating manufacturing capacity

    .  Competing technological and market developments

    .  Changes and developments in collaborative, licensing and other relationships

    .  The development of commercialization activities and arrangements

    .  The leasing and build-out of additional facilities

    .  The purchase of additional capital equipment

We plan to continue our policy of investing available funds in government securities and investment grade, interest-bearing securities, primarily with maturities of one year or less. We do not invest in derivative financial instruments, as defined by Statement of Financial Accounting Standards No. 133.

                                       11

FACTORS THAT MAY AFFECT FUTURE RESULTS

An investment in our common stock involves a high degree of risk. We operate in involves numerous risks and uncertainties. The risks and uncertainties described below are not the only ones we face. Other risks and uncertainties, including those that we do not currently consider material, may impair our business. If any of the risks discussed below actually occur, our business, financial condition, operating results or cash flows could be materially adversely affected. This could cause the trading price of our common stock to decline, and you may lose all or part of your investment. For a more complete discussion of the factors that we currently consider to pose a material risk to our business, please see the section entitled "Factors That May Effect Future Results" in our Annual Report on Form 10-K.

Research and Development/Rapid Growth

A substantial portion of our business plan is based upon the development, production and sale of carbohydrate enzyme therapies for various medical applications. Although we currently have several products at various stages of research and development, none of our enzyme therapies are approved for marketing and sales. All of the products currently in development will require substantial additional research and development including clinical trials prior to distribution and sales.

To be able to effectively address all of the issues associated with developing commercially viable products, we need to continue to develop and improve our research and development capabilities, manufacturing and quality capacities, sales and marketing capabilities, and financial and administrative systems. Our systems, procedures and controls may not be adequate to support our operations and our management may not be able to successfully manage future market opportunities or our relationships with customers and other third parties.

Because the development and manufacture of our enzyme therapy products require specialized technical expertise, the loss of key scientific, technical and managerial personnel may delay or otherwise harm our product development programs. We rely heavily on our ability to attract and retain qualified scientific, technical and managerial personnel. The competition for qualified personnel in the biopharmaceutical field is intense. Our location is in the San Francisco Bay Area, which has a rapidly growing concentration of biopharmaceutical companies, exposes us to particularly intense competition for qualified staff at all levels. We may not be able to continue to attract and retain qualified personnel necessary for the development of our business.

Capital Resources

Developing and bringing our carbohydrate enzyme therapy products to market is a particularly time consuming and capital intensive process which requires substantial expenditures. We believe that the cash, cash equivalents, and short-term investment securities balances at March 31, 2001 will be sufficient to meet our operating and capital requirements through 2001. This estimate is based on assumptions and estimates, which may prove to be wrong. As a result, we will need to obtain additional financing. Such financing may not be available when needed. If we fail to raise additional financing as we need it, we will have to delay or terminate our product development programs.

Regulatory Considerations

We must obtain regulatory approval before marketing or selling our drug products. In the United States, we must obtain FDA approval for each drug that we intend to commercialize. The FDA approval process is typically lengthy and expensive, and approval is never certain. Products distributed abroad are also subject to foreign government regulation. None of our drug products has received regulatory approval to be commercially marketed and sold. If we fail to obtain regulatory approval, we will be unable to market and sell our drug products. Because of the risks and uncertainties in biopharmaceutical development, our drug candidates could take a significantly longer time to gain regulatory approval than we expect or may never gain approval. If regulatory approval is delayed, our management’s credibility, the value of our Company, and our operating results will be adversely affected.

As part of the FDA approval process, we must conduct, at our own and one or more partners (if any) expense, preclinical studies in the laboratory and on animals, and clinical trials on humans for each drug candidate. The

                                       12

number of preclinical studies and clinical trials that the FDA will require will vary depending on the drug product, the disease or condition the drug is being developed to address, the results of prior studies and trials, and regulations applicable to the particular drug. Even if we obtain favorable results in preclinical studies on animals, the results in humans may be different. Results of initial clinical trials on a small number of patients may not predict results on larger clinical trials on a larger number of patients. Adverse or inconclusive clinical results would stop us from filing for regulatory approval of our products.

Typically, if a drug product, such as Aldurazyme, is intended to treat a chronic disease, safety and efficacy data must be gathered over an extended period of time, which can range from six months to three years or more. In addition, clinical trials on humans are typically conducted in three phases. The FDA generally requires two pivotal clinical trials that demonstrate substantial evidence of safety, efficacy and appropriate dosing in a broad patient population at multiple sites to support an application for regulatory approval. If a drug is intended for the treatment of a serious or life-threatening condition and the drug demonstrates the potential to address unmet medical needs for this condition, fewer trials may be sufficient to prove safety and efficacy under the FDA’s Modernization Act of 1997.

Where appropriate, we intend to seek fast track designation from the FDA for our drug candidates. To date, Aldurazyme and rhASB have received a fast track designation. However, a fast track designation does not guarantee a faster review process or a faster approval compared to the normal FDA procedures.

In addition to the risks associated with obtaining regulatory approval for our products, we must comply with strict regulatory requirements relating to the manufacture of our drug candidates that can be costly and could delay or prevent our production efforts. Our manufacturing facilities must obtain regulatory certification prior to production and upon any material change to the production process, before and after product approval, and are continuously subject to inspection by the FDA, the State of California and foreign regulatory authorities. Our Galli Drive and our Bel Marin Keys Boulevard manufacturing facilities have been inspected and licensed by the State of California for clinical pharmaceutical manufacture. We cannot guarantee that these facilities will pass federal or international regulatory inspection. Manufacture of our drug products must comply with the FDA’s current Good Manufacturing Practices regulations, commonly known as cGMP. The cGMP regulations govern quality control and documentation policies and procedures. We cannot guarantee that the Company, or any potential third-party manufacturer of our drug products, will be able to comply with cGMP regulations.

Protection of Intellectual Property

We are dependent on the protection of our intellectual property. We employ several strategies to attempt to prevent our competitors from utilizing our research and technical information. However, these strategies may not be successful.

Where appropriate, we seek patent protection for certain aspects of our technology. Meaningful patent protection may not be available for some of the enzymes we are developing, including Aldurazyme and rhASB. The patent positions of biotechnology companies are extremely complex and uncertain. The scope and extent of patent protection for some of our products are particularly uncertain because key information on some of the enzymes we are developing, including the structure of the enzymes, the methods for purifying or producing the enzymes and the methods of treatment, has existed in the public domain for many years. Publication of this information may prevent us from obtaining composition-of-matter patents, which are generally believed to offer the strongest patent protection.

Even if we seek a patent on an aspect of our technology, obtaining the patent may be difficult or impossible and may require the expenditure of substantial time and money. Competitors may interfere with our patent process in a variety of ways, including claiming that they invented the claimed invention prior to us or that we are infringing on their patents. Competitors may also contest our patents by showing the patent examiner that the invention was obvious or was not original or novel.

Even if we receive a patent, it may not provide much practical protection. If we receive a patent with a narrow scope, then it will be easier for competitors to design products that do not infringe on our patent. Also, enforcing patents is expensive and may absorb significant time by our management. In litigation, a competitor could claim that our issued patents are not valid for a number of reasons. If the court agrees, we would lose that patent.

                                       13

In addition, competitors also seek patent protection for their technology. There are many patents in our field of technology, and we cannot guarantee that we do not infringe on those patents or that we will not infringe on patents granted in the future. If a patent holder believes our product infringes on their patent, the patent holder may sue us even if we have received patent protection for our technology.

In addition to seeking patent protection for our intellectual property, we attempt to protect our trade secrets from disclosure to our competitors. We accomplish this in a number of ways, including limiting access to information to necessary employees and requiring persons with access to trade secrets to enter into nondisclosure agreements.

It is unclear whether our trade secrets will provide useful protection. While we use reasonable efforts to protect our trade secrets, our employees or consultants may unintentionally or willfully disclose our information to competitors. Enforcing a claim that someone else illegally obtained and is using our trade secrets, like patent litigation, is expensive and time consuming, and the outcome is unpredictable. In addition, courts outside the United States are sometimes less willing to protect trade secrets. Also, our competitors may independently develop equivalent knowledge, methods and know-how.

We may also support and collaborate in research conducted by government organizations or by universities. We cannot guarantee that we will be able to acquire any exclusive rights to technology or products derived from these collaborations. If we do not obtain required licenses or rights, we could encounter delays in product development while we attempt to design around other patents or even be prohibited from developing, manufacturing or selling products requiring these licenses. There is also a risk that disputes may arise as to the rights to technology or products developed in collaboration with other parties.

The United States Patent and Trademark Office recently issued a patent that includes claims related to (alpha)-L-iduronidase. Our lead drug product, Aldurazyme, may infringe on this patent. We believe that this patent is invalid on a number of grounds. A patent making the same claims was filed in Europe and has been rejected and cannot be refiled in Europe. Our challenges to the U.S. patent may be unsuccessful, but the rejection of the European application supports our strategy to challenge the validity of the U.S. patent. Even if we are successful, challenging the patent may be expensive, require our management to devote significant time to this effort and may delay commercialization of our product in the United States.

The patent holder has granted an exclusive license for products relating to this patent to one of our competitors. If we are unable to successfully challenge the patent, we may be unable to produce Aldurazyme in the United States unless we can obtain a sub-license from the current licensee. The current licensee is not required to grant us a license and even if a license is available, we may have to pay substantial license fees and royalties, which could adversely affect our business and operating results.

Orphan Drug Status

As part of our business strategy, and as a further means of protecting our intellectual property, we intend to develop certain drugs that may be eligible for FDA and European Community orphan drug designation. Under the Orphan Drug Act, the FDA may designate a product as an orphan drug if it is a drug intended to treat a rare disease or condition, defined as a patient population of less than 200,000 in the United States. The company that obtains the first FDA approval for a designated orphan drug for a given rare disease receives marketing exclusivity for use of that drug for the stated condition for a period of seven years. However, different drugs can be approved for the same condition. Similar regulations are available in the European Community with a ten-year period of market exclusivity.

Because the extent and scope of patent protection for our drug products is limited, orphan drug designation is particularly important for our products that are eligible for orphan drug designation. We plan to rely on the exclusivity period under the orphan drug designation to maintain a competitive position. If we do not obtain orphan drug exclusivity for any one of our drug products and we are unable to otherwise protect the product, our competitors may then sell the same drug to treat the same condition, following their own filing with and approval by the FDA.

                                       14

We received orphan drug designation from the FDA for Aldurazyme for the treatment of MPS-I in September 1997. In February 1999, we received orphan drug designation from the FDA for rhASB for the treatment of MPS-VI. In February 2001 we received orphan drug designation from the European Community for both products. Even though we have obtained orphan drug designation for these drugs and even if we obtain orphan drug designation for other products we develop, we cannot guarantee that we will be the first to obtain marketing approval for any orphan indication or that exclusivity would effectively protect the product from competition. Orphan drug designation neither shortens the development or regulatory review time of a drug so designated nor gives the drug any advantage in the FDA review or approval process.

Issues Relating to Our Joint Venture

We have entered into a joint venture with Genzyme Corporation to assist us in obtaining international regulatory approval for Aldurazyme as well as marketing and commercializing the product worldwide. We are relying on Genzyme to apply the expertise it has developed through the launch and sale of Ceredase® and Cerezyme® enzymes for Gaucher disease, a rare genetic disease. Because it is our initial product, our financial results and market value are substantially dependent upon the development of Aldurazyme.

Genzyme may not devote the resources necessary to successfully gain regulatory approvals internationally and to market Aldurazyme worldwide. In addition, either party may terminate the joint venture for specified reasons, including if the other party is in material breach of the agreement or has experienced a change of control or has declared bankruptcy and also is in breach of the agreement.

If the joint venture is terminated, one party, as determined by the joint venture agreement, must buy out the other party’s interest in the joint venture and will then own all rights to Aldurazyme. If Genzyme were obligated to buy out our interest in the joint venture, Genzyme would be granted, exclusively, all of the rights to Aldurazyme and any related intellectual property and regulatory approvals. We would then effectively be unable to develop and commercialize Aldurazyme. If we were obligated to buy out Genzyme’s interest in the joint venture, we would then be granted all of these rights to Aldurazyme exclusively. While we could then continue to develop Aldurazyme, that development may be slowed because we would have to divert substantial capital to buy out Genzyme’s interest in the joint venture and would have to search for a new partner to commercialize the product and to obtain foreign regulatory approvals or to develop these capabilities ourselves.

Termination of the joint venture where we retain the rights to Aldurazyme could cause us significant delays in product launch in the United States, difficulties in obtaining third-party reimbursement and delays or failure to obtain foreign regulatory approval, any of which could hurt our business and results of operations. Since Genzyme funds 50% of the joint venture’s operating expenses, the termination of the joint venture would double our financial burden and reduce the funds available to us for other product programs until such time (if any) that we were to enter into another partnership with a third party.

Complicated Manufacturing Process

Even once we have successfully developed a product and obtained regulatory approval for its sale and use, there are still several factors that could limit or prevent its commercial viability including large scale manufacturing complications, distribution and marketing, and market demand.

We have developed approximately 41,200 square feet at our Novato facilities for the manufacturing of Aldurazyme and rhASB. We expect that the manufacturing process of all of our new products, including rhASB, will require significant time and resources before we can begin to manufacture them in commercial quantity with appropriate cost.

Except for Aldurazyme,we have no experience manufacturing recombinant human enzymes in volumes that will be necessary to support commercial sales. The large scale, consistent production of several of our enzymes is complicated, expensive and unpredictable and may not yield the high quality and high purity required with acceptable quantity and costs. Improvements in manufacturing processes typically are very difficult to achieve and are often very expensive. We cannot know with certainty how long it might take to make improvements if it became necessary to do so. If we contract for manufacturing services with an unproven process, our contractor is subject to the same uncertainties, high standards and regulatory controls.

                                       15

If we do not achieve our manufacturing cost targets, we will have greater losses in manufacturing start-up phases and lower margins and reduced profitability in commercial production. Even if we can establish the necessary capacity, we cannot be certain that manufacturing costs will be commercially reasonable, especially if third-party reimbursement is substantially lower than expected.

Marketing and Pricing Issues

Our initial drug candidates target diseases with small patient populations. As a result, our prices must be high enough to recover our development costs and achieve profitability and the payers for the patient populations must be able to reimburse the prices of the treatments. For example, two of our initial drug products in genetic diseases, Aldurazyme and rhASB, target patients with MPS-I and MPS-VI, respectively. We estimate that there are approximately 3,400 patients with MPS-I and 1,100 patients with MPS-VI in the developed world. We believe that we will need to market worldwide to achieve significant market share. In addition, we are developing other drug candidates to treat conditions, such as other genetic diseases and serious burns, with small patient populations. We cannot be certain that we will be able to obtain sufficient market share for our drug products at a price high enough to justify our product development expenses.

The course of treatment for patients with MPS-I using Aldurazyme and MPS-VI using rhASB is expected to be expensive. We expect patients to need treatment throughout their lifetimes. We expect that most families of patients will not be capable of paying for this treatment themselves. There will be no commercially viable market for Aldurazyme or rhASB without reimbursement from third-party payers.

Third-party payers, such as government or private health care insurers, carefully review and increasingly challenge the price charged for drugs. Reimbursement rates from private companies vary depending on the third-party payer, the insurance plan and other factors. Reimbursement systems in international markets vary significantly by country and by region, and reimbursement approvals must be obtained on a country-by-country basis. Third-party payers may not be willing to pay for the costs of our drugs and the courses of treatment at reimbursement rates that will be enough to allow us to profit from sales of our drugs.

We currently have no expertise obtaining reimbursement. We expect to rely on the expertise of our partner Genzyme to obtain reimbursement for Aldurazyme. We will need to develop our own reimbursement expertise for future drug candidates unless we enter into collaborations with other companies with the necessary expertise.

We expect that in the future, reimbursement will be increasingly restricted both in the United States and internationally. The escalating cost of health care has led to increased pressure on the health care industry to reduce costs. Governmental and private third-party payers have proposed health care reforms and cost reductions. A number of federal and state proposals to control the cost of health care, including the cost of drug treatments have been made in the United States. In some foreign markets, the government controls the pricing, which affects the profitability of drugs. Current government regulations and possible future legislation regarding health care may affect our future revenues from sales of our drugs and may adversely affect our business and prospects.

                                       16

Item 3.     Quantitative and Qualitative Disclosure about Market Risk.

The Company’s exposure to market risk for changes in interest rates relates primarily to the Company’s investment portfolio. The Company places its investments with high credit issuers and by policy limits the amount of credit exposure to any one issuer. As stated in its policy, the Company seeks to improve the safety and likelihood of preservation of its invested funds by limiting default risk and market risk. The Company has no investments denominated in foreign country currencies and therefore is not subject to foreign exchange risk.

The Company mitigates default risk by investing in high credit quality securities and by positioning its portfolio to respond appropriately to a significant reduction in a credit rating of any investment issuer or guarantor. The portfolio includes only marketable securities with active secondary or resale markets to ensure portfolio liquidity.

The table below presents the carrying value for the Company’s investment portfolio. The carrying value approximates fair value at March 31, 2001.

Investment portfolio:
                                                  Carrying value
                                                 (in $ thousands)
                                                 ----------------

Cash and cash equivalents.......................... $ 19,722
Short-term investments.............................   10,044*
                                                     --------
   Total........................................... $ 29,766
                                                    ==========

* 100% in United States agency securities.

                                       17

                           PART II. OTHER INFORMATION

Item 1.           Legal Proceedings.                                       None.

Item 2.           Changes in Securities and Uses of Proceeds.


In February  2001,  we issued 25,000 shares of common stock to Fredric D. Price,
our  Chief  Executive  Officer  and  Chairman  of  the  Board,  pursuant  to his
employment  agreement  with us. The shares  issued were pursuant to an exemption
from registration  under Section 4(2) of the Securities Act of 1933, as amended.
The shares are  appropriately  legended to  indicate  that the shares may not be
resold  unless  registered  under  the  Securities  Act  or  an  exemption  from
registration is available for such sale.


Item 3.           Defaults upon Senior Securities.                         None.

Item 4.           Submission of Matters to a Vote of Security Holders.     None.

Item 5.           Other Information.                                       None.

Item 6.           Exhibits and Reports on Form 8-K.

(a)       The following documents are filed as part of this report

                            See Exhibit Index attached hereto.

                   (b)  Reports on Form 8-K.

                        No reports were filed on Form 8-K during the
                        three months ended March 31, 2001.

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                                  EXHIBIT INDEX


Exhibit
Number         Description of Document
- -------        ------------------------------
3.1            Registrant's Articles of Incorporation and Bylaws
10.1           License Agreement between BioMarin Pharmaceutical Inc. and
               W.R. Grace & Co., effective as of January 1, 2001

                                       19
     

                                    SIGNATURE

         Pursuant to  the requirements of  the Securities Exchange Act of 1934,
the registrant caused this Report to be signed on its behalf by the undersigned,
 thereunto duly authorized.




                               BIOMARIN PHARMACEUTICAL INC.


Dated: May 4, 2001         By:/s/ Raymond W. Anderson
- ------------------            --------------------------------------------
                              Raymond W. Anderson
                              Chief Financial Officer, Chief Operating
                              Officer, Secretary and V.P. Finance and
                              Administration (on behalf of registrant
                              and as principal financial officer)

                                       20