• Cholelithiasis is a common complication of homozygous sickle cell disease. The frequency of gallstones appearing in patients with heterozygous sickle hemoglobinopathies is unknown. We performed sonographic tests on 65 unselected patients with major sickle hemoglobinopathies. Cholelithiasis was found in 11 (26%) of the 42 patients with hemoglobin SS, in three (20%) of the 15 with Hb SC, and in one (12.5%) of the eight with Hb S-β-thalasssemia. There was a correlation between the presence of gallstones and increasing age. All patients with major sickle hemoglobinopathies are at risk for the development of biliary tract disease.