[Cholangiocarcinoma associated with Caroli's disease. Apropos of a case. Review of the literature].

This is a report of a rarely observed occurrence of complication in Caroli's disease, i.e. the emergence of a cholangiocarcinoma in the course of the disease. There are 20 observations reported in the literature, and the hypotheses of pathogenesis set forth are reviewed. The frequency of a malignant transformation during the course of Caroli's disease is compared to the frequencies of other hepatic-biliary congenital cystic tumours. The excision of the cyst resulted in an appreciable period of survival for the patient.