Caroli's disease is a rare entity that is included in the fibropolycystic abnormalities of the bile ducts. Ultrasonographic patterns consist of evident dilatation of the bile ducts. Although it is thought to be a congenital disease, it usually presents in young adults and few cases have been reported in children. We present the case of a 10-year-old boy with Caroli's syndrome (Caroli's disease, congenital hepatic fibrosis and polycystic renal disease). Evolution was favorable.