Eleven total knee replacements were performed in eight patients with severe haemophilia A and the patients were followed up for two to eight years. All the patients had disabling haemophilic arthropathy of one or both knees, which had not responded to conservative treatment. Postoperative complications occurred in 10 knees, including nose bleeding, haemarthrosis, anaphylactic reactions, urinary tract infection with haematuria, recurrent phlebitis at infusion sites, and fever for a few days. There were no wound infections. The outcome, as determined by a standard scoring system, was rated as excellent or good in nine knees, fair in one and poor in one. Nevertheless, all patients were free of pain and all but one returned to full-time or part-time employment. Total knee arthroplasty appears to be a satisfactory procedure in the treatment of disabling haemophilic arthropathy of the knee.