Cystic disease of the biliary tree is a rare and heterogeneous medical condition that may be diagnosed during intrauterine life or can become symptomatic with cholestatic jaundice or acute recurrent pancreatitis before adulthood. The etiology remains obscure although abnormalities of the pancreaticobiliary junction may be the primary pathology in some, with biliary dilatation following reflux of activated pancreatic secretions. Pancreaticobiliary maljunction may however remain asymptomatic up to adulthood without or with minimal choledochal dilatation, but carries a relevant risk of biliary carcinogenesis. The modified Todani classification, which is based on anatomical characteristics, is the current standard classification method of biliary cystic malformation. Most anomalies will present in childhood with obstructive jaundice or abdominal pain; however, the classical triad of pain,jaundice and palpable mass is not common. Complete cyst excision, where possible, and biliary reconstruction are the treatment for most types. In case of biliary cystic disease associated with ductal plate malformation, the therapeutic solution must be more radical and these patients are candidate to liver transplant.