The company said that Vimizim was reviewed under the Orphan Drug program and it is the first treatment approved in Australia for Morquio A syndrome, an ultra-rare, severely debilitating disease affecting about 3,000 patients in the developed world.
Australia MPS & Related Diseases Society National manager Nicole Millis said: "Treatment for Morquio A syndrome has been lagging behind for a number of years, leaving patients with few options that could really make a difference in their daily lives.
"We are grateful for BioMarin’s commitment to the MPS community and for providing a therapy to these patients."
In February, the US Food and Drug Administration (FDA) approved the Vimizim license application to treat patients with Morquio A syndrome.
Vimizim is also approved in Canada and the European Union, while marketing applications have been submitted in several other countries.
BioMarin chief medical officer Hank Fuchs said: "The approval of Vimizim in Australia underscores our relentless commitment to providing this much needed therapy to patients with Morquio A syndrome across the globe.
"We will continue to leverage our expertise and dedicate our resources to advancing therapies within the MPS community, as well as other communities with ultra-rare conditions, to ensure patients get the treatments they deserve."