NCPF has been classically described as a disease of adulthood with a mean age of onset in third to fourth decade.1 Though reported in more than one-third of cirrhotic patients, HPS is a rare entity in non-cirrhotic portal hypertension and is usually asymptomatic, especially in younger patients.2-4 Very limited pediatric data are available for HPS prevalence, especially in non-cirrhotic subjects..