Personal experience in the treatment of a patient, presenting a pure unilobar Caroli's disease, is described. According on the litterature data, the procedure of choice was the left hepatectomy. The main reason was the high incidence of malignant transformation to cholangiocarcinoma of the cells of the cystic walls. Up to now the follow-up of the patient seems to prove that it was the right indication. Other procedures are discussed.
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