Gallbladder disease is known to be increased in sickle cell disease (SS). The prevalence of cholelithiasis in children with SS has been reported as 10% to 37%. Gallbladder ultrasound, cholecystography, and/or abdominal radiography were used to evaluate 31 patients aged 2 to 18 years with no systematic evidence of gallbladder disease for cholelithiasis, Overall prevalence of gallstones was found to be 29%. Cholelithiasis was detected in children as young as 4 years of age. Prevalence below the age of 10 years and above the age of 10 years was found to be 11% and 55% respectively. The increased prevalence of cholelithiasis in children with sickle cell disease suggests the use of diagnostic ultrasonography as a routine screening procedure.