The results of primary total knee replacement performed on a group of haemophiliac patients in a single institution by the same surgeon using the same surgical technique and prosthesis are reported. A total of 35 primary replacements in 30 patients were carried out between 1996 and 2005 and were reviewed retrospectively. The mean age of the patients was 31 years (24 to 42) and the mean follow-up was for 7.5 years (1 to 10). There were 25 patients with haemophilia A and five with haemophilia B. The HIV status and CD4 count were recorded, and Knee Society scores determined. Two patients had inhibitors to the deficient coagulation factor. There were no early wound infections and only one late deep infection which required a two-stage revision arthroplasty, with a good final result. The incidence of infection in HIV-positive and negative patients was thus similar. One knee in a patient with inhibitor had excessive bleeding due to a pseudoaneurysm which required embolisation. The results were excellent in 27 knees (77%), good in six (17%) and fair in two (6%). The survival rate at 7.5 years taking removal of the prosthesis for loosening or infection as the end-point was 97%. The mechanical survival of total knee replacements in haemophiliacs is very good. Our results confirm that this is a reproducible procedure in haemophilia, even in HIV-positive patients with a CD4 count > 200 mm. 3. and those with inhibitors. Our rate of infection was lower than previously reported. This could be due to better control of the HIV status with highly active anti-retroviral therapy and the use of antibiotic-loaded cement