The authors describe a case of orthotopic liver transplantation (OLT) in a patient with an infantile form of choledochal cyst that progressed to severe hepatic failure. Choledochal cyst must be included in the differential diagnosis of extrahepatic biliary obstruction in infants; it represents an additional reason to consider early exploratory laparotomy, both for diagnosis and to perform definitive excision. In the event that end-stage liver disease develops, OLT should be considered.