Choledochal cysts are rare pathological conditions characterized by dilatation of the intrahepatic and extrahepatic bile ducts. Postulated etiologies are an abnormal pancreato-biliary junction favoring reflux of pancreatic juice to the biliary tree, and aganglionosis of the biliary tree. They have a wide range of presenting symptoms, the main ones being abdominal pain, jaundice and evident abdominal mass. Choledochal cysts are premalignant lesions, and therefore the treatment of choice is complete surgical resection, which demands long-term follow-up.