INTRODUCTION: Gastrointestinal hemorrhage is a common complication among cirrhotic patients with portal hypertension, due to the development of bleeding varices. While esophagus and stomach are the most common location for development of varices, it can also rarely occur in ectopic locations, such as the jejunum, ileum, rectum and duodenum. Here, we report a case of a patient with severe gastrointestinal hemorrhage due to a bleeding jejunal varix. CASE DESCRIPTION/METHODS: A 55-year-old male with a history of hypertension, splenectomy, and alcoholic cirrhosis had presented initially to an outside ED with complaints of bright red blood per rectum. At admission, he required multiple infusions of pRBCs, and several upper endoscopies due to refractory bleeding with unclear source. Initial endoscopies showed non-bleeding esophageal varices, for which he underwent ligation banding. However, he continued to deteriorate, developing recurrent melena, hypotension, and syncope. On admission to our hospital, he had a repeat upper endoscopy, lower endoscopy, CT angiography and tagged red blood cell study; but we were unable to localize the source of the bleeding. He continued to deteriorate, and also developed a PEA arrest in the setting of a profuse bright red blood per rectum, requiring massive transfusion protocol. After successful resuscitation, he underwent a small bowel enteroscopy, which finally revealed a bleeding jejunal varix. However, the varix was not amenable to any endoscopic intervention. Subsequently, he underwent a successful balloon-occluded retrograde transvenous obliteration (BRTO) of varix. Patient’s condition stabilized and after adequate recovery, he was extubated. DISCUSSION: Early diagnosis of ectopic varices is difficult to achieve due to low likelihood of presentation and low sensitivity to various diagnostic tests. Due to its low prevalence, there are no protocols for its management. As in our patient, endoscopic intervention such as sclerotherapy and band ligation can be difficult due to the narrow lumen of the small bowel. Alternatively, BRTO and TIPS are also considered effective therapeutic options. However, due to his poor liver function, BRTO was a better alternative. In conclusion, through this case report, we want to emphasize the importance of early recognition and appropriate management of ectopic varices due to its potential for massive, even fatal hemorrhage.