Esophageal varices are the most common site of variceal bleeding. However, bleeding from varices that are not located in the esophagus may account for up to 30% of all variceal bleeding. Significant varices can occur in the stomach, duodenum, jejunum, ileum, colon, rectum, and biliary tree. They can also occur at the site of a surgical ostomy. These types of varices bleed less commonly than esophageal varices, but they can also be far more difficult to diagnose and treat. The absence of stigmata of recent esophageal variceal bleeding and certain clues in the patient's history and clinical presentation should raise the clinician's suspicion of an extraesophageal site of variceal bleeding. Particularly, patients with extrahepatic causes of portal hypertension, cirrhotic patients with a prior history of gastrointestinal surgery, and patients who present with profound bleeding but without hematemesis need to be evaluated further if an obvious site of esophageal variceal bleeding is not seen on initial endoscopy. In this article, we review the features particular to extraesophageal varices as well as the diagnosis and management of bleeding from these varices. We use the term extraesophageal rather than ectopic because the term ectopic varices implies exclusion of both esophageal and gastric varices.