Introduction: Cirrhosis is a disease causing morbidity and mortality in children. Children affected present with growth failure and complications of liver disease such as abdominal pain, fatigue, hepatosplenomegaly , and jaundice. Primary sclerosing cholangitis (PSC) is a rare cause of cirrhosis in young children, as it usually presents during the second decade of life. There is no medical cure and liver transplant remains the only option. Only a few rare cases were published in the literature with only 1 patient being younger than our patient. Case report: A 4 year old adequately grown boy , presented with haematamesis with no past history suggestive of chronic liver disease. He was pale and had no peripheral stigmata of chronic liver disease. The liver appeared shrunken on percussion with 3cm splenomegaly . Investigations revealed an Hb of 5g/dl with normal liver function tests except for elevated GGT and ALP. USS abdomen showed evidence of portal hypertension with cirrhotic architecture of the liver. Upper Gastro Intestinal Endoscopy revealed grade 2 esophageal varices. Liver biopsy showed features of primary sclerosing cholangitis with cirrhosis. After the acute management he was started on propanalol and the parents were counselled with the possible need of liver transplant in future. Discussion: Though children with cirrhosis present with symptoms and signs of chronic liver disease and growth failure, this patient had neither. PSC is rare in early childhood. It is important find the exact cause of cirrhosis in children as the management can be planned and explained to the parents early .