Congenital hepatic fibrosis (CHF) is an uncommon genetic disorder characterized by periportal fibrosis with irregularly shaped proliferating bile ducts, intrahepatic portal hypertension, and esophageal varices. CHF is associated with a disability of renal functions, normally caused by an autosomal recessive polycystic kidney disease, which is a serious kind of polycystic kidney disease. The patient is born with this disorder (inborn), and it is inherited as an autosomal recessive trait. The normal liver abnormalities are an expanded liver, increased force in the venous structure that carries blood from distinct organs to the liver.

Fiber-like connective tissue that spreads over and through the liver (hepatic fibrosis), frequently referred to as hepatic lesions. Gastrointestinal (abdomen and intestines) bleeding is often an earlier signal of this circumstance. Affected individuals too have impaired renal role, normally caused, in children and teenagers. CHF too is associated with cholangitis. Commonly, the hepatic lesion is associated with renal participation characterized by cystic tubular dilatations affecting both the cortical and medullary portions of the kidney. The longer the patient survives, the less distinctive the renal pathology becomes.

Medical therapy is provided mainly in the presence of cholangitis. Portosystemic shunt operation is the handling of selection for Congenital hepatic fibrosis (CHF) circumstance. Prophylactic consumption of sclerotherapy is yet contentious. Liver transplant too is considered in the administration of CHF complicated by recurrent cholangitis or bankruptcy to react to respective medical and postoperative curative modalities resulting in liberal hepatic dysfunction. Sclerotherapy is indicated for the handling of intense bleeding from esophageal varices and as a main therapy for administration of recurrent or chronic variceal hemorrhage.