Sickle cell anemia is an inherited hemoglobinopathy in which there is a structural change to the erythrocyte from round to crescent shaped or sickled. These abnormally shaped cells can block small vessels resulting in compromise of vascular supply, pain, and end-organ damage. These patients are particularly susceptible to hip osteonecrosis, which in late stages may require a total hip arthroplasty. Historically, total hip arthroplasty had inferior outcomes in this patient population due to poorer clinical outcomes and higher perioperative complications. During recent decades, however, there have been some improvements in the medical management of these patients, which has potentially improved the clinical outcomes of this procedure. In this article, we review all reported management strategies for the perioperative care of sickle cell anemia patients undergoing total hip arthroplasty.