SummaryThe observation of a patient with Caroli’s disease, i.e., congenital dilatation of the intrahepatic bile ducts is reported. The profound abnormalities of the biliary tree, observed long before the initial description of the disease, were the source of the initial clinical picture which led to surgery. The surgical procedure which included a cholecystectomy together with a choledocoduodenostomy was followed by a number of episodes of relapsing cholangitis, two of whom required further surgical drainage of the common bile duct and intrahepatic biliary tree. The diagnostic approach and the surgical procedure (Roux-en-Y hepaticojejunostomy) performed twenty years later are described and discussed with reference to an updated review of the literature concerning this uncommon condition.