Summary. The outcomes of total knee arthroplasty (TKA) and total hip arthroplasty (THA) in patients with haemophilia have not been compared with other patient populations. The aim of this study was to compare the results of joint replacement therapy in patients with and without haemophilia retrospectively. This is a controlled retrospective cohort study. The complications and long-term results of 21 TKAs and 6 THAs performed in 22 haemophilia patients were compared with those of 42 TKAs and 12 THAs in patients without bleeding disorders. Patients were matched for type of arthroplasty, gender, year of surgery and age. Blood loss, infection rate, revision, implant survival and function as judged by the patient were recorded. Haemarthrosis occurred in 14 (52%) of the 27 arthroplasties performed in the haemophilia patients, while four bleedings were recorded in the 54 arthroplasties in the control group (7%, P < 0.001). All bleeds occurred in TKAs. In the patient group, two infections (7%, both in TKAs) occurred compared to seven (13%, 6/7 in TKAs) in the control group (NS). In the haemophilia patients, all but one (96%) arthroplasties were still in situ at the end of follow-up, vs. 44 (81%, NS) in the control group. For TKAs, survival was 20/21 vs. 34/42 respectively (P = 0.25). Subjective function was good in 22/27 (81%; 76% in TKAs) arthroplasties in haemophilia patients, vs. 40/54 (74%; 71% in TKAs) in controls. Haemophilia patients experienced significantly more haemarthroses, but no more infections and they have an excellent implant survival compared with non-haemophilia controls.