Idiopathic portal hypertension (IPH) is a very rare disease, characterized by portal hypertension with obstruction or stenosis of the intrahepatic peripheral portal branches, in the absence of liver cirrhosis or obstruction of hepatic vein and main portal vein. This condition also has other denominations such as idiopathic non-cirrhotic portal hypertension and non-cirrhotic portal fibrosis. The etiology and pathogenesis of IPH remain unclear, and may be related to heredity, immunity, infection and other factors. The main manifestations of this disease are hypersplenism and gastroesophageal varices, with less occurrence of hepatic insufficiency and hepatic encephalopathy. There are still many controversies in the diagnosis and treatment of IPH. Its diagnosis is only made by exclusion, and treatment chiefly refers to the guidelines for cirrhotic portal hypertension. There are few data on prognosis of IPH, and IPH is considered to have a better prognosis than that of cirrhotic portal hypertension, and in only a few patients may progress to liver failure.