[Malignant Caroli's disease. Etiopathogenic hypotheses, apropos a case]

We present a case report of Caroli's disease with related cholangiocarcinoma in a 30 years old white woman. The risk of Caroli's disease malignization is estimated about 7-12% being cholangiocarcinoma the histopathologic pattern most frequently found. This frequency, which is over 100 times higher than that expected in the general population, make us think over the possibility of being some premalignant agent, if not a really carcinogenic one in Caroli's disease. The risk of degeneration is proportional to time of evolution of the disease so, when it is possible, the resective surgery should be elected.