Choledochal cyst refers to congenital dilatation of the main biliary route associated or not with the segmental intrahepatic ducts. We report the case of a female patient, 40 years old, with the diagnosis of choledochal cyst, who at 17 years of age had undergone surgical treatment. She evolved with cholangitis of repetition and obstructive jaundice. The imaging exams showed dilation of the biliary intra-hepatic routes, mainly on the left, hepatolithiasis, and a large cystic lesion in the liver pedicle. The laparotomy evidenced that the previous treatment had been a cholecystectomy and cyst draining through Roux cystojejunostomy in Y, which was blocked. The treatment consisted of resection of the choledochal cyst, hepatectomy of the 2 and 3 segments, and Roux hepaticojejunostomy in Y. The patient is in the fourth year of outpatient follow-up with no complications. On the basis of its association with cancer, resection and not just draining is the recommended treatment of choledochal cyst.
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