[Congenital hepatic fibrosis associated with choledochal cyst].

The purpose of this work is to report the case of a one-year and eleven-month-old female child who presented with an asymptomatic hepatic mass; on x-ray examination a well limited round tumor was seen as well as renal cysts. A choledochal cyst was found upon laparotomy; the liver biopsy exhibited congenital hepatic fibrosis. This association is not clearly defined in the literature review made, making this perhaps the first well-documented case with this combination. We analyze the pathogenic mechanisms of both disorders and conclude that choledochal cyst is probably not related to hepatorenal fibropolycystic disease; this could be a casual association in our patient.