[Congenital hepatic fibrosis: a report of a case in a child with Down's syndrome].

An association of congenital hepatic fibrosis and Down syndrome in an 8-year-old boy is described. The child presented a form of congenital hepatic fibrosis with portal hypertension, splenomegaly and platelets loss. In the patient's family there are no manifestations of hepatic and renal disease.