Cholelithiasis is an unusual complication of hemolytic uremic syndrome. A 12-year-old boy with hemolytic uremic syndrome, established by renal biopsy, who developed cholestatic jaundice is presented here. Laboratory results for secondary causes of hemolytic uremic syndrome were normal. Abdominal ultrasonography and magnetic resonance cholangiopancreatography revealed extrahepatic obstruction. A common bile duct stone, discovered by retrograde cholangiopancreatography was extracted by sphincterotomy. In conclusion, cholelithiasis should be considered as a cause of abdominal pain and cholestasis in patients who are diagnosed as having hemolytic uremic syndrome.