A case of a 70 year old man who was found to have an extrahepatic portal vein aneurysm during an evaluation for hematuria is reported. Extrahepatic portal vein aneurysms are rare with only twenty cases reported in the literature. Typically, patients present with hemorrhage requiring surgical exploration or the aneurysm is discovered during evaluation of another abdominal process. Management includes careful follow-up in the asymptomatic patient without underlying liver disease or portal hypertension.
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