AbstractIn 1958, Caroli [1, 2] was the first to describe congenital dilatations of intrahepatic bile ducts concerning a segment, a sector, a lobe, or both halves of the liver. Sometimes the dilatations are associated with congenital hepatic fibrosis as described in 1954 by Grumbach [3], dilatations of biliary ductules (biliary angiomatosis), and parenchymal sclerosis. This hereditary autosomal recessive disease is associated in many cases with nephrospongiosis or Cacchi‐Ricci's disease [4].Caroli's disease is quite rare. By 1982, a total of 99 cases had been reported in the world literature. Aside from these cases we have seen and operated on 10 patients [5, 6].In Caroli's disease, stenosis and dilatations give rise initially to biliary stasis and, secondly, to intrahepatic biliary lithiasis and biliary infection. The normal course in Caroli's disease consists of suppurative cholangitis, septicemia with gram‐negative organisms, and intrahepatic and subphrenic abscesses. The disease must be recognized before these serious complications arise. The new hepatobiliary imaging procedures should now allow an early diagnosis and, therefore, a better therapeutic approach.