Abstract: Idiopathic portal hypertension (IPH) is a rare disorder causing portal hypertension, clinically characterized by features
of portal hypertension, normal liver functions, which is not associated with hepatic vein and portal vein obstruction. Infections and
thrombotic predisposition constitute the likely pathogenesis in Eastern and Western patients, respectively. Hepatic vein pressure
gradient (HVPG), spleen/liver stiffness ratio and liver biopsy can be used to differentiate IPH from liver cirrhosis. IPH is relatively
benign in nature. Portal thrombosis is an independent predictor of poor outcome. Management centers on the prophylaxis of variceal
bleeding, which is similar to that of cirrhosis. Anticoagulation therapy and surgical shunts must be considered in some portion of IPH
patients.