Duodenal variceal bleeding is an uncommon complication of portal hypertension that can easily go unrecognized and reach mortality rates as high as 40%. Cirrhosis is the most common cause of duodenal varices. In most cases, duodenal varices occur concomitantly with esophageal varices, further complicating identification with initial endoscopy. Although many modalities have been explored with respect to management and treatment approaches, guidelines have yet to be established owing to the infrequency in which bleeding occurs from ectopic duodenal varices. We present a case of massive duodenal variceal hemorrhage that highlights the complexity of initial diagnosis and ultimately required a transesophageal intrahepatic portosystemic shunt with coil embolization for control of bleeding.