We report two cases of extrahepatic portal vein aneurysm, and both of them underwent surgical intervention. The first case had a mild pain in right upper quadrant of the abdomen; the second had no obvious symptoms. Physical examination revealed nothing abnormal. Both of them were diagnosed by magnetic resonance imaging angiography (MRA). One of the aneurysms was located at the main portal vein, the other, at the confluence of the superior mesenteric vein and the splenic vein, and these two places are exactly the most common locations of the extrahepatic portal vein aneurysm reported in the literature (30.7% each site). The first case underwent aneurysmorrhaphy and the second case, aneurysm resection with splenectomy. Both of them recovered soon after the operation, and the symptom of the first case was greatly alleviated. During the follow-up of half a year, no complication and adverse effect of surgical intervention was found and the color Doppler ultrasonography revealed no recurrence of the aneurysmal dilation. We suggest that surgical intervention can alleviate the symptom of the extrahepatic portal vein aneurysm and prevent its complications effectively and safely for low risk patients.