Portal vein aneurysms are extremely rare, less than 200 cases have been reported until late 2015. They are defined as a portal vein diameter exceeding 19 mm for cirrhotic patients and 15 mm in normal livers. Most patients are asymptomatic, but complications may occur. We report a case of a 68-year-old female admitted for etiological assessment of a portal hypertension revealed by an upper gastro intestinal bleeding, who was incidentally diagnosed with a portal vein aneurysm.