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With reference to a patient with Caroli's disease (segmentally dilatated intrahepatic bile ducts), the clinical features of this rare but serious disease are described. The condition differs from other cystic dilatations of the bile ducts in that only the intrahepatic bile ducts are involved. Its pathogenesis is obscure. It is suspected that an embryonic developmental disorder in the wall of the bile ducts causes cysts via physiological periodical increases of pressure. The diagnosis is made accidentally by cholangiography via the T-drain during or after cholecystectomy. When Caroli's disease is suspected, echography can be a valuable diagnostic aid.
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