Total hip replacement was performed in 27 hips of patients who had sickle cell anaemia with avascular necrosis of the femoral head. The disease was bilateral in 11 patients. Considerable medical problems were encountered although most of the patients had exchange transfusion before surgery (86%), which prevented postoperative sickle cell crises in all but two cases. At the primary operation hard sclerotic bone was seen in nine femora with complete obliteration of the femoral canal. There were four femoral fractures, three following perforation of the shaft due to this hard bone. There was a very high morbidity due to loosening in both cemented and uncemented prostheses. With a rate of 59% over a cumulative 5.5 year period, revision was being performed at an average of only 43 months. Surgeons should be aware of these problems.