In children, cholelithiasis is relatively rare and frequently result from some etiologic factors such as hemolytic disease, anatomic malformation of the biliary tract and disturbance of the enterohepatic circulation of bile salts, especially in infants. Seven cases with cholelithiasis have been treated in our department during the 13-year-6-month period between April 1, 1972 and October 31, 1985. Of these cases, 3 were associated with congenital biliary dilatation, one with hereditary spherocytosis and 2 in siblings with familial intrahepatic cholestasis. The remaining one was diagnosed on autopsy incidentally, in a patient with omphalocele and died of aspiration pneumonia. Cholelithiasis associated with familial, intrahepatic cholestasis is very rare, only four cases have been reported previously in the world. One hundred and twenty nine cases, including our 7 cases, have been collected in Japanese previous literatures. These cases were evaluated with regard to causes, clinical symptoms, diagnostic methods, etc. Recently, reported cases of cholelithiasis in children have gradually increased and were diagnosed mostly by ultrasonography, because of this examination is easy and not invasive.
