Human immunodeficiency virus-infected hemophiliacs are at risk for bacterial and opportunistic infections with worsening immunosuppression. Thus, the risk of postoperative infection following orthopaedic surgery is of considerable concern. A survey of United States hemophilia treatment centers was conducted to determine the incidence of postoperative infection in human immunodeficiency virus-positive hemophiliacs with CD4 counts of 200 mm3 or less undergoing orthopaedic surgery. A total of 115 centers from 37 states reported that postoperative infection occurred in 10 (15.1%) of 66 patients undergoing 74 orthopaedic procedures, between several weeks and 5 months following surgery. In five (50%), pre-operative infection preceded postoperative joint infection. Staphylococcus was the most common organism isolated in a prosthetic joint infection, in 6 of 10 (60.0%), and the knee was the most commonly affected joint, in 9 of 10 (90.0%). Joint arthroplasty appeared to have 10 times the risk of nonarthroplasty procedures for postoperative infection (9 of 34 [26.5%] and 1 of 40 [2.5%], respectively, P < .01). Two subjects developed chronic osteomyelitis. The rate of postoperative infection in human immunodeficiency virus-positive hemophiliacs with CD4 counts of 200/mm3 or less appears to be high, when compared with the general population. Early, vigorous treatment should be instituted for suspected infection, antibiotic prophylaxis considered for invasive procedures, and surgical intervention individualized based on the balance of risks and benefits.