An increase in the incidence of abnormalities on bone and mineral metabolism (osteopenia/osteoporosis) and the development of osteonecrosis has been observed in patients with acquired immunodeficiency syndrome (AIDS). Two cases of osteonecrosis in patients with AIDS are reported. Both patients were receiving highly active antiretroviral therapy (HAART) and presented with one or more known risk factors for osteonecrosis. We review the literature and discuss the pathogenesis, diagnosis, prevention and treatment of this entity in patients with AIDS.