Twenty-three total knee arthroplasties in 15 patients with severe hemophilia were performed between February 1974 and September 1988. Thirteen patients had Factor VIII deficiency and two had Factor IX deficiency. The mean followup period was 7.5 years, with a minimum of 4 years for patients who were alive (eight) at the time of this review. Seven patients had died before this report, and all were seropositive for the human immunodeficiency virus. Using the Hospital for Special Surgery knee scoring system, the result was excellent in one knee, good in three, fair in two, and poor in 17. One patient was seropositive for the human immunodeficiency virus at the time of the index procedure, and 12 were seropositive at the most recent followup; the human immunodeficiency status of three patients was unknown. There were two early and two late deep infections, all in patients who were seropositive for the human immunodeficiency virus. The most recent postoperative radiographs for all knees were reviewed using the Knee Society radiographic scoring system. Ten femoral components were well fixed, 11 were possibly loose, and two were probably loose. Eight tibial components were well fixed, 10 possibly loose, three probably loose, and two definitely loose. One knee had been revised for aseptic loosening. There are few published studies of the long term results of total knee arthroplasties in patients with hemophilia. In this series of 23 knees, there was a high rate of loosening and infection. Total knee arthroplasty may be a useful treatment for the relief of pain attributable to end stage hemophilic arthropathy, but there is a high rate of complications, especially in patients who are seropositive for the human immunodeficiency virus.