Background: SCA causes chronic haemolysis which is a risk factor for cholelithiasis. Objective: To determine the prevalence and outcome of children with SCA complicated with gallstones treated at the sickle cell clinic at the children emergency hospital Khartoum state. Methods: 261 patients age 4 months to 16 years were studied. AUS examination was carried out. The 30 patients in whom gall stones were detected followed prospectively from June 1996 to September 2009 when a second AUS examination was obtained.Results: Gall stones occurred in 30 patients of whom four were lost to follow up in the first year. The overall prevalence of cholelithiasis was 11.5% and it increased with age. The youngest patient with cholelithiasis was 2 1/2 years old. Haematological variables, bilirubin and sex did not identify a subgroup of patients at higher risk for gallstones. All the patients were asymptomatic at the time of diagnosis. One patient developed symptoms 3years after the diagnosis and he was submitted to surgery. The 25 remaining asymptomatic patients were followed up for 13 years and none of them presented complications related to cholelithiasis during this period. Conclusion: The prevalence of cholelithiasis in Sudanese children and adolescents with SCA was significant. The large majority patients remained asymptomatic over a long period. African Health Sciences 2013; 13(1): 154 -159 http://dx.doi.org/10.4314/ahs.v13i1.21