OBJECTIVES:  To estimate the overall health system costs of treating haemophilia A (HA) in Australia, using recombinant factor VIII (rFVIII), ADVATE. METHODS:  A literature-based cost study for treatment of people with severe HA in the absence of inhibitors was undertaken. ADVATE treatment was assumed to be given prophylactically to prevent bleeding episodes or "on-demand' for a bleeding episode. Utilisation data for rFVIII were derived from the Australian Bleeding Disorders Registry. Total health system costs were calculated by summing the cost of rFVIII (prophylaxis and "on-demand" use), and other direct medical costs for treating bleeding episodes and any adverse events. All costs are reported in AUD2016. RESULTS: Australian data were available for only five identified variables associated with treating HA: cost per international unit of rFVIII; cost of some adverse events; rFVIII usage by haemophilia severity and treatment regimen; and cost of orthopaedic surgeries. Australian data were unavailable for important variables such as hospitalisations from bleeds, home therapy, orthopaedic procedures, joint abnormalities, and median annualised bleed rates, all of which are necessary to derive costs from a health system perspective. Utilising Australian data and supplementing international data where necessary, approximate health system costs for rFVIII ADVATE patients in the absence of inhibitors receiving prophylaxis treatment was AU$92,000 (AU$83,000 rFVIII treatment cost) and "on-demand" was $38,000 (AU$31,000 rFVIII treatment cost) per severe HA patient per annum. The population cost per annum was approximately AU$51,612,000 (AU$45,966,000 rFVIII treatment cost) for 640 severe HA patients in Australia receiving treatment in 2014/15. CONCLUSIONS:  Utilising Australian cost data supplemented with international data, finds the cost of rFVIII (ADVATE) is reasonable for a very effective treatment in preventing and/or treating bleeding episodes and comparable to other government funded highly specialised and/or life-saving drugs in Australia. Further research into the societal cost and benefits of treating HA in Australia is warranted.