Herein, we presented a case of Caroli's syndrome associated with medullary sponge kidney and reviewed the literature. A 21-year-old male was admitted to our internal medicine department with the complaints of fatique, anorexia and recurrent right upper abdominal pain. Abdominal ultrasonography showed hepatomegaly and multiple dilated intrahepatic bile ducts. Computed tomography of the abdomen indicated cystic images distributed in both lobes of the liver and multiple medullary cysts within the kidneys. The magnetic resonance cholangiopancreatography (MRCP) pointed out multiple cystic dilatations of intrahepatic bile ducts. The main bile duct and extrahepatic bile ducts were appeared to be normal. The patient had a brother who had been diagnosed of Caroli's syndrome 12 years ago. Radiologic investigation of the other family members revealed no abnormality.