Objective To investigate diagnosis and surgical management of femoral head osteonecrosis in sickle cell disease(SCD). Methods The related literature available today was reviewed, and the clinical advance in the diagnosis of femoral head osteonecrosis complicating SCD and its surgical management was evaluated. Results Although the advance in the diagnosisand the surgical treatment for the femoral head osteonecrosis in SCD had been achieved, challenging clinical problems still remained because of many complications after the surgical treatment. Whether the surgical management was reasonablein a particular stage of the disease and for a specific patient still required investigation. Conclusion Whether the salvage of the patient’ship joint should be performed or the artificial joint replacement should be performed still needs a cautious consideration before either of the surgical managements is given.