Abstract Background Superior mesenteric arteriovenous fistula is a rare vascular anomaly often presenting with sequelae of portal hypertension, heart failure, or mesenteric ischemia. This report describes a patient with a previously unidentified superior mesenteric arteriovenous fistula who presented with variceal bleeding, thought to be the leading cause of mortality associated with this condition. Although this patient was initially referred for a transjugular intrahepatic portosystemic shunt procedure, following a thorough review of her clinical history and imaging, she instead underwent embolization of the arteriovenous fistula likely responsible for her symptoms. Case Presentation A 75-year-old woman with a past surgical history of ext...