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Sickle cell disease (SCD) is an autosomal recessive disorder that results in hemolytic anemia related to abnormal hemoglobin and erythrocyte levels. SCD is characterized by vascular occlusive episodes, visceral sequestration, and aplastic or hemolytic crises. These crises most commonly occur in bone. The orthopaedic manifestations of SCD comprise much of the morbidity associated with this disorder. Osteonecrosis and osteomyelitis are among the most disabling and serious musculoskeletal complications in patients with SCD. Effective management of the bone and joint sequelae requires an accurate diagnosis, an understanding of the pathophysiology of the disease, and knowledge of available medical and surgical treatment alternatives. The major orthopaedic manifestations of SCD are osteonecrosis, osteomyelitis, septic arthritis, and bone infarction. Patients with SCD require close monitoring in the perioperative period because of the risk for vasoocclusive crisis.