Aneurysmal malformation of the portal vein is a rare entity. To our knowledge, only scattered reports ofportal vein aneurysms appear in the literature in English, and there is no previously published report in Korean.We describe a case exhibiting aneurysmal malformation of the extrahepatic portal vein at the hepatic hilum; thefindings demonstrated by ultrasound, CT and angiography are discussed, a review of previously described cases isincluded.