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We report a rare case of refractory gastrointestinal bleeding due to portal hypertension caused by a gastroduodenal artery-superior mesenteric vein fistula. A 54-year-old woman with a 6-year history of haematemesis and haematochezia was admitted after her most recent bleeding episode. She had endured over 10 episodes of gastrointestinal bleeding, undergone multiple endoscopic procedures and received one percutaneous transhepatic variceal embolisation. One week before admission, she experienced another bleeding episode, necessitating an emergency transjugular intrahepatic portosystemic shunt (TIPS). Six days post-TIPS, she returned with recurrent haematochezia. Preoperative CT revealed significant thickening and dilation of the superior mesenteric vein, indicating a possible fistula, which was confirmed through coeliac trunk angiography. We successfully occluded the fistula, leading to a reduced portal vein pressure gradient and the resolution of her symptoms. Portal hypertension resulting from an arteriovenous fistula is uncommon, and the closure of the fistula is essential for effective treatment.