Arterioportal fistulas (APFs) are rare vascular communications between the mesenteric arterial tree and the portal vein. The causes and presentations of this entity are varied. APFs related to tumor, infection, and trauma have been described. Patients may be asymptomatic or present with hemobilia, portal hypertension, or mesenteric steal syndrome. Small APFs require no intervention, with treatment indicated for large or symptomatic APFs. The treatment has shifted from surgical ligation or hepatic resection to an endovascular-first approach. We describe an endovascular treatment option for the management of an acquired extrahepatic (type 2) APF and present a review of the literature.