Choledochal cyst is a congenital or acquired anomaly affecting the biliary tree in which exists a dilatation of the bile duct, not only the choledochus is affected but also the intrahepatic and extrahepatic ducts might be affected. The clinical presentation is not specific, even the classic triad with abdominal pain, mass, and jaundice is not common as suspected, found only in 10% of cases. Clinicians must rely on imaging studies for diagnosis and classification. The treatment is cyst excision with hepaticoenterostomy in most of the cases, but in some others, a liver transplant would be necessary. These patients require lifelong follow-up due to its rate of recurrence compared with general population.