A case of cystic congenital dilatation of hepatocholedoch, in a patient aged 27, is presented. The malformation affects the entire biliary duct, being classified as Todani Ic type. The diagnosis was intraoperatively established and the surgical solution was the quasitotal extirpation of the hepatocholedoch followed by the reconstruction of the biliary transit through hepaticojejunostomy on Y loop Roux. The postoperative evolution was favourable and the result is still good after 13 years.
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