The purpose of this study was to evaluate the results of shoulder arthroplasty for the treatment of avascular necrosis in patients with sickle cell disease. Medical records, radiographs, operative reports, and outcome scores of 8 adult patients with sickle cell disease were evaluated. The mean follow-up was 51 months (range, 2-10 years). Seven patients had a hemiarthroplasty, and one had a total shoulder arthroplasty. One patient had an intraoperative rotator cuff tear. Two had sickle cell crises in the immediate postoperative period. In one patient, stiffness developed that required arthroscopic capsular release 22 months after her arthroplasty. Another patient with a hemiarthroplasty underwent revision to a total shoulder arthroplasty 5 years after the index procedure. The mean American Shoulder and Elbow Surgeons score improved by 31.9 points. However, only 2 patients reported improvement in pain as assessed with a visual analog scale. Although shoulder arthroplasty provides improvements in range of motion and function in the majority of patients, pain relief is less predictable.