Vachel and Stevens were the first to describe a case of cystic dilatation of the intrahepatic bile ducts in 1906, but Jacques Caroli in 1958 gave a more thorough description of a syndrome of congenital malformation of the intrahepatic ducts with segmental cystic dilatation, increased biliary lithiasis, cholangitis and liver abscesses, associated with renal cystic disease or tubular ectasia. He described it as “nonobstructive saccular or fusiform multi-focal segmental dilatation of the intra-hepatic bile ducts”; basically, he observed cavernous ectasia in the biliary tree causing a chronic, often life-threatening hepatobiliary disease. ^[1]