Choledochal cyst is a rare developmental malformation of the biliary tree and has serious problem of transformation to malignancy. The development of cholangiocarcinoma related to choledochal cyst increases as the age of patient increases and is more common in Orientals with female predominance of 2.5 times. Prevalence rate of cholangiocarcinoma is various from 2.5 to 15.6% in adult with choledochal cyst, but very low in children. We experienced a case of cholangiocarcinoma with multiple liver metastases arising from type I choledochal cyst in a 13-year-old girl who complained of sudden onset of right upper quadrant abdominal pain.