Congenital bile duct cyst are a rare condition in adults and in western countries, but the disease is gaining terrain due to the increasing institutional referral and the improved means of non-invasive diagnostic tools. Todani type V bile duct cyst is described as solitary or multiple intrahepatic dilatations. Congenital nonparasitic hepatic cysts could communicate with biliary tree, making difficult the differentiation simple cyst and diverticular dilatation of the bile branches. We present the case of a woman with a large cyst communicating with the duct of segment VI which was developed mostlyextrahepatic, putting real problems of diagnostic and therapy. Although these two entities (Todani V choledochal cysts and hepatic congenital cysts) are separately classified, they have the same mechanism of development, the ductal plate malformation, which requires a reform of the current classification of congenital hepato-biliary cysts. [ABSTRACT FROM AUTHOR]