8-K

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                       SECURITIES AND EXCHANGE COMMISSION

                             WASHINGTON, D.C. 20549





                                    FORM 8-K


                                 CURRENT REPORT

                     Pursuant to Section 13 or 15(d) of the
                         Securities Exchange Act of 1934


                Date of Report (Date of earliest event reported):
                         September 6, 2001 (September 6, 2001)


                          BioMarin Pharmaceutical Inc.
             (Exact name of registrant as specified in its charter)




         Delaware                  000-26727                68-0397820
(State or other jurisdiction      (Commission     (IRS Employer Identification
    of incorporation)              File Number)                No.)



     371 Bel Marin Keys Boulevard,                            94949
              Suite 210                                     (Zip Code)
          Novato, California
        (Address of principal
          executive offices)


                                 (415) 884-6700
              (Registrant's telephone number, including area code)











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Item 5.           Other Events

On September 6, 2001, we announced the completion and the preliminary results of
our Phase I clinical trial of recombinant human N-acetylgalactosamine
4-sulfatase (rhASB) for the treatment of MPS VI in a press release, dated
September 6, 2001. A copy of the press release is attached hereto as Exhibit
99.1 and incorporated herein by reference.



Item 7.               Financial Statements and Exhibits

EXHIBIT NO.   DESCRIPTION OF DOCUMENT
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99.1          Press Release Dated September 6, 2001 related to completion of
              MPS VI Phase I Trial.





                                   SIGNATURES

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


                            BioMarin Pharmaceutical Inc.


                            By: /s/ Raymond W. Anderson
                               -------------------------------------------------
                               Raymond W. Anderson
                               Chief Operating Officer, Chief Financial Officer,
                               Secretary and Vice President Finance and
                               Administration

Date:  September 6, 2001

                                  Exhibit Index

EXHIBIT NO.   DESCRIPTION OF DOCUMENT
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99.1          Press Release Dated September 6, 2001 related to completion of
              MPS VI Phase I Trial.

EX-99

NEWS

Contacts:
Fredric D. Price                                     Sharon Karlsberg
Chairman and Chief Executive Officer                 Director
BioMarin Pharmaceutical Inc.                         Feinstein Kean Healthcare
(415) 884-6700                                       (617) 577-8110

Investor Relations
(415) 884-6777

For Immediate Release:

            BioMarin Announces Positive MPS VI Phase I Trial Results

              Safety and Biochemical Activity of rhASB Demonstrated
                in Patients with Life-threatening Genetic Disease

     Company Plans to Initiate a Phase II Trial in the First Quarter of 2002

Novato, CA, September 6, 2001 - BioMarin Pharmaceutical Inc. (Nasdaq and SWX New
Market: BMRN) today announced that recombinant human N-acetylgalactosamine
4-sulfatase (rhASB), an investigational enzyme replacement therapy for the
treatment of mucopolysaccharidosis (MPS) VI, was well tolerated at both dose
levels by the six patients evaluated in the Company's Phase I clinical trial,
and there were no drug-related serious adverse events during the study.
Furthermore, rhASB reduced levels of urinary glycosaminoglycans (GAGs), a
complex carbohydrate, in all six patients as predicted by preclinical studies
conducted in MPS VI-affected cats.

Trial Results

The trial's primary objective was to evaluate the safety of rhASB, and the
enzyme was well tolerated by all six patients. Key findings included:

        o  No drug-related serious adverse events;
        o  No significant allergic reactions to the enzyme infusions;
        o  Urinary GAG excretion was reduced by a mean of 70% and 55% in the
           high and low dose groups, respectively;
        o  Reduced urinary GAG excretion was evident within three weeks of
           initiating treatment.

The reduction in urinary GAGs demonstrates that rhASB is breaking down the
complex carbohydrate materials that otherwise accumulate in patients with MPS VI
and lead to the debilitating and life-threatening symptoms of the disease.

Paul R. Harmatz, M.D., Associate Director, Pediatric Clinical Research Center,
Children's Hospital Oakland, the trial's principal investigator, said, "The six
MPS VI patients enrolled in this trial represented a broad array of clinical
manifestations and severity of the disease. I was pleased to see clinical
improvements as the trial progressed and believe rhASB has potential as a
treatment for patients with MPS VI."

Dr. Harmatz will present data from the trial at the American Society of Human
Genetics annual meeting in San Diego on Sunday, October 14th, 2001.

All six patients continue to receive therapy and will be evaluated at 24-week
intervals.

Stuart J. Swiedler, M.D., Ph.D., BioMarin's Vice President, Clinical Affairs,
commented, "The trial successfully met its primary objective of demonstrating
the safety of treatment with rhASB, and, as a result, we are planning to
initiate a Phase II trial of rhASB early next year. Clinical product for this
larger trial will be produced in our recently expanded clinical manufacturing
facility located in Novato."

In October 2000, BioMarin initiated a randomized, double-blind Phase I clinical
trial at Children's Hospital Oakland to evaluate the safety, efficacy, and
pharmacokinetics of weekly intravenous infusions of rhASB at two dose levels as
an enzyme replacement therapy in six MPS VI patients. All six patients received
the enzyme therapy, but the patients, the medical staff involved in the trial,
and BioMarin were blinded as to whether the patients received a high or low
dose.

More about MPS VI

MPS VI (also known as Maroteaux-Lamy Syndrome) is a debilitating,
life-threatening genetic disease for which no drug therapies are currently
available that is caused by a deficiency of the enzyme N-acetylgalactosamine
4-sulfatase. The deficiency leads to the accumulation of GAGs in the lysosomes,
the digestive organs of the cell. This accumulation in the lysosomes leads to
progressive cellular, tissue and organ system dysfunction. Debilitating symptoms
can include impaired cardiac and pulmonary function, delayed physical
development, skeletal and joint deformities, impaired vision and hearing, sleep
apnea, and reduced endurance. The majority of patients die from disease-related
complications between childhood and early adulthood, depending on the severity
of the disease.

BioMarin has received orphan drug and fast track designations for rhASB from the
FDA. In addition, the European Commission has designated rhASB for the treatment
of MPS VI as an orphan medicinal product in the European Community.

BioMarin specializes in the development and commercialization of therapeutic
enzyme products.

This press release contains forward-looking statements about the business
prospects of BioMarin Pharmaceutical Inc., including forward-looking statements
regarding the prospects, development, commercialization and regulatory
approval of rhASB. These forward-looking statements are predictions and involve
risks and uncertainties such that actual results may differ materially from
these statements. Results may differ materially depending on the progress of
BioMarin's product programs, the results of current and proposed clinical
trials, actions of regulatory authorities, future actions in the pharmaceutical
market, developments by competitors, and those factors detailed in BioMarin's
filings with the Securities and Exchange Commission such as 10Q, 10K and 8K
reports. Stockholders are urged not to place undo reliance on forward-looking
statements, which speak only as of the date hereof. BioMarin is under no
obligation, and expressly disclaims any obligation, to update or alter any
forward-looking statement, whether as a result of new information, future events
or otherwise.