Congenital hepatic fibrosis (CHF) is a rare autosomal recessive disorder characterized by ductal plate malformation of the intrahepatic bile ducts, leading to progressive periportal fibrosis and portal hypertension. It is considered part of the fibropolycystic disease spectrum and frequently coexists with renal anomalies, particularly autosomal recessive polycystic kidney disease (ARPKD). The pathogenesis involves mutations in the PKHD1 gene, which encodes fibrocystin/polyductin, a protein essential for ciliary function and biliary morphogenesis. Clinically, CHF presents with hepatosplenomegaly, esophageal varices, and recurrent cholangitis, though some cases remain asymptomatic into adulthood. Histopathological features include abnormal bile duct proliferation, hypoplastic portal vein radicles, and bridging fibrosis without nodular regeneration. Diagnosis relies on imaging modalities such as ultrasound and MRI, supported by liver biopsy for definitive confirmation. Management is primarily supportive, targeting complications of portal hypertension and cholangitis. Liver transplantation may be indicated in advanced cases.