Total joint replacement (TJR) is an option for the management of chronic haemophilic arthropathy. Because surgery is technically challenging, there is a high rate of deep prosthetic infections, particularly in human immunodeficiency virus (HIV)-infected individuals. We determined the incidence of deep infection rates following total knee and hip arthroplasties in HIV-seropositive and HIV-seronegative persons with haemophilia. Fifty-one primary joint replacements were performed on 32 patients seen at a regional comprehensive haemophilia care center from 1975 to 2002. Thirty prostheses were placed in patients who were HIV-seropositive prior to surgery (n = 14) or seroconverted later (n = 16). Median age at the time of surgery was 33 years (range: 20-61) among 19 HIV-seropositive patients and 35 years (range: 26-74) among 13 HIV-negative patients. Median duration of follow-up was 83 months (range: 2-323). Rate of primary joint infection per artificial joint-year by HIV status was compared by Poisson regression. Main outcome measures were the incidence of primary replacement joint infections by HIV status. Deep infections developed in five (9.8%) of 51 replacement joints. There were two infections during 204.15 joint-years without HIV infection and three infections during 205.28 joint-years with HIV infection. The incidence rate of joint infection (0.98 vs. 1.46 per 100 joint-years) was not increased with HIV (relative risk, RR: 1.49, 95% CI: 0.25-8.93, P = 0.66). We conclude that HIV infection is not a contraindication to knee or hip replacement arthroplasty in the appropriate clinical setting.