Variceal bleeding is a life-threatening complication of portal hypertension in patients with cirrhosis. Varices are portosystemic venous shunts that arise as a result of portal hypertension, most commonly located in the gastroesophageal (GE) area. Ectopic varices (ECV) are enlarged portosystemic venous collaterals located anywhere outside of the GE region. ECV bleeds are uncommon but can lead to significant morbidity and mortality. We present a rare case of lower GI bleeding from a small bowel varix in a J-pouch. A 34-year-old female with a history of ulcerative colitis status post total proctocolectomy with ileal pouch-anal anastomosis (IPAA or J-pouch) and primary sclerosing cholangitis with compensated cirrhosis was transferred to our hospital for an overt, obscure GI bleed. She initially presented to an outside hospital (OSH) with hematochezia and syncope. EGD, push enteroscopy, pouchoscopy, and capsule endoscopy at the OSH all failed to find the source of bleeding. Due to continued overt bleeding requiring 14 U of packed RBCs over 10 days, she was transferred to our medical center. Repeat EGD found no blood and no gastroesophageal varices. Pouchoscopy revealed red blood and clots in the J-pouch, but normal underlying mucosa and no obvious source of bleeding. A tagged RBC scan was positive for bleeding localized to a loop of small bowel in the right lower quadrant. CT-angiogram demonstrated a large ectopic venous collateral adjacent to the IPAA (Fig. 1) which was supplied by the superior mesenteric vein and drained by the right gonadal vein (Fig. 2), consistent with a small bowel varix. This ECV was the most likely cause of her recurrent bleeding. She underwent TIPS with reduction of portosystemic gradient from 25 to 11 mmHg and significant decompression of the ECV, rendering selective embolization unnecessary. She was discharged 5 days later and had no further bleeding at a 2 month follow-up visit. ECV account for 1-5% of all variceal bleeding episodes. Small bowel varices are most commonly seen in patients with portal hypertension and prior abdominal surgery. A triad of portal hypertension, hematochezia without hematemesis, and previous abdominal surgery should prompt evaluation for small bowel varices via CT scan with both arterial and venous phases. This case illustrates that, although rare, small bowel varices can present as massive lower GI bleeding and require a multidisciplinary approach with therapy tailored based on institutional expertise.Figure: CTA venous phase showing large venous collateral adjacent to J-pouch consistent with small bowel varix.Figure: Venogram illustrating enlarged superior mesenteric vein (SMV) filling varix with outflow tract through the right gonadal vein (R Gonadal V.).