Congenital intrahepatic biliary duct dilatation associated with congenital hepatic fibrosis (CHF), referred to as Caroli's syndrome, is a rare condition. Caroli's syndrome is generally associated with autosomal recessive polycystic kidney disease (ARPKD) or rarely autosomal dominant polycystic kidney disease (ADPKD).in this case report, we describe a thirteen and a nine- year-old two brothers with Caroli's syndrome and polycystic kidney disease. There was no parental consanguinity. The elder brother had a history of jaundice. However, the little one had only a history of periodic abdominal pain and on follow up, he had acute pyelonephritis.