Abstract: Objective The aim of this study was to report a case of patients with idiopathic portal hypertension(IPH) and review the related literatures. Methods One patients with IPH was reported, and the studies on LT for IPH were retrieved from MEDLINE, EMBASE, Wanfang data and other databases. The outcome after LT in patients with IPH was analyzed. Results A 57-year-old female was clinically diagnosed as liver cirrhosis(LC), underwent LT due to gastrointestinal bleeding and ascites, and the final diagnosis was IPH based on the liver histopathology examination; the percutaneous liver biopsy was performed at the third year of follow-up and showed the nodular regenerative hyperplasia(NRH), with mild portal inflammation and fibrosis, suggesting recurrence of IPH; a total of 81 patients with IHP were retrieved from the literatures, and forty-two patients were diagnosed as LC before LT; the longest follow-up period after LT was 248 months and eight patients died; five patients underwent liver biopsy 3.5 month to 14 years after the first LT, and the pathology showed NRH again; among them, 3 patients developed NRH with portal hypertension after LT in 7th month, 3 years and 14 years, respectively. Conclusions The patients with IPH with severe portal hypertension or liver failure should receive LT, and a few patients might have "recurrence" of IPH, which warrants further investigation.

Key words: Idiopathic portal hypertension, Liver transplantation, Prognosis, Recurrence