Jacques Caroli for the first time described in the year 1958, cavernous dilatation of the hepatic bile ducts. It's the rare abnormality; there have been reported less than 200 cases until now. The disease is caused by defects in the genetic program which are transmitted in an autosomal recessive way. There are two basic types of the disease: focal (so called. "simple type") and diffuse, embracing entire bile tree. Both types maybe associated with congenital hepatic fibrosis and then the abnormality is usually called "Grumbach disease". There is an excessive risk of cancer (cholangiocarcinoma) in patients with Caroli's disease. Dominant symptoms are due to recurrent cholangitis, sometimes with signs of portal hypertension. Basic treatment is hepatic resection (focal form) and internal bypass of the biliary tree (diffuse form). The prognosis of the disease is reserved one.