“Idiopathic non-cirrhotic portal hypertension” is a rare diagnosis characterised by increased intrahepatic portal pressures in the absence of underlying liver disease and caused by an unexplained aetiology. We present a one-of-a-kind “case of a 16-year-old male” with recurrent variceal bleeding who had an “elevated hepatic venous pressure gradient” and “portal hypertension sequelae” without underlying liver illness. There is limited treatment available because management is mostly geared at preventing illness consequences. This example emphasises the importance of future investigation into this disease entity and its pathophysiology.