Although the incidence of biliary carcinoma associated with choledochal cyst is high, there are extremely rare reports about cancer development in the remaining intrapancreatic biliary tract in patients who underwent primary excision of the choledochal cyst. The authors present a case of carcinoma associated with the remnant intrapancreatic biliary tract in a 39 year-old woman, 17 years after the initial excision of a choledochal cyst with Roux-en-Y hepaticojejunostomy. Long-term follow-up may be recommended even in patients who have undergone excisional surgery for choledochal cyst, because it is possible that cancer associated with the intrapancreatic biliary tract may develop.
 

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