Congenital cysts of the biliary tract are rare anomalies generally observed in pediatric age, exceptionally in adults. The different extension and the morphology of cystic lesions make possible a classification in subtype. This disease, of Len asymptomatic, is characterized by high incidence of complications such as pancreatitis, cholangitis, and cancer. For these reasons, congenital cystic dilatation of bile duct should be radically treated by complete resection of the dilated extraepatic biliary tract. A review of the International Literature and a rare case of congenite dilatation of the intra and extraepatic biliary ducts in a female 54 years old, treated by choledochal resection with hepatico-jejunostomy on Roux en Y segment, are presented.