A 37-year-old male patient presented with abdominal pain and diarrhea. Computed tomography showed a large superior mesenteric vein aneurysm. The patient had a history of Crohn's disease and underwent an ileocecal resection 7 years previously. A selective angiogram of the superior mesenteric artery revealed that a dilated branch of this artery fed directly into the superior mesenteric vein. The iatrogenic superior mesenteric arteriovenous fistula was successfully closed by transarterial coil embolization. Successful endovascular treatment for a superior mesenteric arteriovenous fistula has been recently reported; however, the complications of this new modality are not well understood. We herein review the current literature and discuss endovascular treatment.