A 20-year-old man with congenital choledochal dilatation associated with carcinoma was treated. At the age of 10 years he underwent a cholecystectomy and partial resection of the cyst. At admission the patient complained of hypochondriac and back pain combined with bilious vomiting. He underwent a palliative operation. A review of the literature is presented on treatment and prognosis of patients with carcinoma arising in choledochal congenital dilatation previously treated by surgery other than excision. It is emphasized that the cystic dilatation should be removed at the time of the first operation. Furthermore, in patients who underwent surgical treatment without excision of the choledochal dilatation, the dilated biliary tract should be removed even in absence of subjective symptoms.