Abstract: Objective To investigate the etiology， clinical symptoms， diagnosis， and treatment of idiopathic non-cirrhotic portal hypertension （INCPH）， combined with a literature review. Methods We retrospectively analyzed the clinical data of 1 case of idiopathic non-cirrhotic portal hypertension，combined with domestic and foreign literature for analysis. Results The patient was admitted to the hospital with ″ hematemesis for one day″ and was misdiagnosed as cirrhotic portal hypertension. Portal hypertension predated the onset of decompensated liver cirrhosis based on the patient′s medical history. Therefore，the diagnosis was considered idiopathic non-cirrhotic portal hypertension，but the treatment method has always been the same as that of cirrhotic portal hypertension. The 10-year survival rate of patients is higher than that of cirrhotic portal hypertension. Conclusion Idiopathic non-cirrhotic portal hypertension is a rare disease characterized by no liver cirrhosis，no other causes of liver disease，and no visceral vein thrombosis with portal hypertension. There are currently no treatment guidelines for INCPH，and expert opinions recommend following the prevention and treatment guidelines for patients with cirrhosis and portal hypertension. Idiopathic non-cirrhotic portal hypertension is mainly based on preventing and treating complications. Patients with INCPH have a better prognosis than cirrhotic portal hypertension.

Key words: idiopathic portal hypertension, liver cirrhosis, portal hypertension, esophageal varicose veins