Twenty-five total knee arthroplasties were performed in 21 patients with hemophilia. The mean patient age was 35.8 years and mean follow-up time was 6.2 years. The average preoperative knee score increased from 18.6 points (range, 3-29) to 82.8 points (range, 44-99). The average preoperative knee function score increased from 41.4 points (range, 20-60 points) to 75.8 points (range, 45-95 points). The average preoperative range of motion was 73.4 degrees with an average flexion contracture of 22.6 degrees, whereas the average postoperative range of motion increased to 92.2 degrees with an average flexion contracture of 5.6 degrees. Median consumption of coagulation factor concentrate decreased from 4837 U/month before operation to 1500 U/month 1 year after surgery. The total knee arthroplasty is a useful treatment in severe hemophilic arthropathy to obtain pain relief and functional improvement, and to reduce the need for ongoing treatment using coagulation factor concentrate.