A case of sickle cell (HbSS) disease is presented in a patient with a history of recurrent admissions for abdominal pain, jaundice, and abnormal liver function tests. Although he was believed to have a sickle cell abdominal crisis, his abdominal X-ray films revealed three calcified stones. Each of these stones progressively passed through the common duct and into the duodenum while awaiting surgery. He has been followed for two years since his cholecystectomy without further hospitalizations.

This case led to the investigation of cholelithiasis in sickle cell disease to dispel the following misconceptions. Some physicians and pediatricians believe that (1) cholelithiasis and cholecystitis are uncommon in sickle cell disease; (2) the complications of gallstones are not significant; (3) the operative risk in patients with sickle cell disease is high; (4) these patients with HbSS disease do not live long enough to get into trouble with gallstones.

A review of the literature on cholelithiasis and HbSS disease presents adequate evidence to cause us to urge investigation of the gallbladder in all patients with HbSS disease and abdominal crises, and cholecystectomy as an elective procedure should stones be present.

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