BACKGROUND Extrahepatic portal venous aneurysms (PVAs) are rare, and the pathogenesis is not fully understood. The optimum management of these patients is unknown. METHODS Consecutive patients with PVA were identified over an 18-year period (1992-2010). A retrospective review was conducted. Clinical presentation, modality of diagnosis, surgical treatment, 30-day morbidity and mortality, and follow-up are reported. RESULTS Four patients were identified who underwent surgical management of an extrahepatic PVA. Operative technique using left renal vein, femoral vein panel graft, polytetrafluoroethylene (ePTFE) graft, and segmental aneurysm wall resected with aneurysmorrhaphy is described. Early complications occurred in 1 patient with an ePTFE graft. The patient returned to the operating room for bleeding. In addition, the same patient had a late graft thrombosis 6 years postoperatively when the anticoagulation was discontinued for pregnancy. The remainder of the patients recovered without complication, and their repairs are still patent with a mean follow-up of 78 months (17-144 months). There were no mortalities in the series. CONCLUSIONS Operative intervention for portomesenteric venous aneurysm can be done safely in select patients and should be considered in those with symptoms, rapid growth, mural thrombus, or aneurysms ≥4 cm in diameter. Repair with an autogenous interposition graft affords good long-term patency. Aneurysmorrhaphy may be performed if the remaining venous wall is of good quality.