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Unusual case of digestive hemorrhage: celiac axis-portal vein arteriovenous fistula. A case of intractable upper gastrointestinal-hemorrhage was reported in a patient with portal hypertension caused by an arterioportal fistula (APF), namely, celiac axis-portal vein arteriovenous fistula. Portal hypertension caused by extrahepatic-APFs is extremely rare. Trauma, malignancy, and hereditary causes are the common etiology of APFs; but were absent in our patient. Our patient represents an unusual case of unexplained APF who presented with portal hypertension and was successfully managed through endovascular aortic repair.