Avascular necrosis, which most commonly affects the femoral head, is a well-recognized entity in orthopedic surgery. Maxillofacial surgeons have recently recognized that a similar process may also be at work in the temporomandibular joint because of the involvement of the mandibular condyle. Avascular necrosis of the mandibular condyle in association with sickle cell anemia is an infrequent finding. In this report, a patient with avascular necrosis of the mandibular condyle in the late stage causing fibrous ankylosis of the temporomandibular joint in sickle cell anemia is presented.