According to their experience and to a review of the most important papers in the field, the Authors examine the clinical, morphological, diagnostical and therapeutical aspects of congenital hepatic fibrosis. The initial sign is almost always hepatosplenomegaly and diagnosis can be achieved by means of clinical, anamnestic, histological and ecotomographic findings. Relationships among congenital hepatic fibrosis, infantile polycystic disease of the kidney and liver and Caroli's disease are stressed.