Personal experience in the treatment of a female patient presenting a pure monolobar Caroli's disease, is described. The woman was asymptomatic so far; during the last 2 weeks she was admitted on 3 occasions with repeated attacks of cholangitis and obstructive jaundice. Surgery was performed for relief of the jaundice. A diagnosis of segmental Caroli's disease (congenital dilatation of intrahepatic bile ducts) with congenital fibrosis was made on the basis of marked fibrous septa with the characteristic ductal plate formation on left hepatectomy specimen and the cysts seen on ultrasound.