Caroli’s disease is a rare inherited disorder characterized by nonobstructive saccular or fusiform dilation of the intrahepatic bile ducts. Two main types of Caroli’s disease are reported, the true type with isolated dilation of intrahepatic bile ducts of single liver segment with formation of stones and development of cholangitis. The second type is combined with a congenital hepatic fibrosis. The true type of Caroli’s disease is distinct from primary sclerosing cholangitis, polycystic disease, cholangiolithiasis and other diseases. This paper presents a literature review and a case of monolobar Caroli’s disease with intrahepatic stones. The difficulties of preoperative differential diagnosis are shown.
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