Abstract
This case report presents an unusual case of cholangiocarcinoma arising nearly 35 years after cystoduodenostomy for choledochal cyst. The patient visited our hospital with dyspepsia and studies revealed bezoar within the choledochal cyst caused by bile and food reflux. The patient underwent pancreaticoduodenectomy and a biopsy revealed adenocarcinoma, stage IIB. After 19 months, the patient has no recurrence to date and has recovered well. This case shows that proper surgical management and meticulous, long-term follow-up is imperative for patients with congenital choledochal cyst.