Gallstones are frequently found in patients with sickle cell anemia. The differentiation between acute calculous biliary tract disease and sickle cell crisis can be difficult and should be based on the clinical presentation, comparison with previous episodes of abdominal pain, and judicious use of hepatobiliary radionuclide scanning. Emergency cholecystectomy is associated with a high morbidity and should be avoided if possible. Elective cholecystectomy is associated with a lower but still significant risk of complications. We believe patients with sickle cell anemia and symptomatic cholelithiasis should have elective cholecystectomy. Careful management is essential to minimize the danger of postoperative complications.