Introduction. Arteriovenous fistula of the superior mesenteric blood vessels is a rare complicaton in abdominal surgery. Case report. We presented a 49-year-old man with cramplike abdominal pain, abdominal distension and weight loss symptoms, with a history of previous small bowel resection and right colectomy, due to Crohn disease, 16 years ago. Clinical examination revealed a paraumbilical pulsation with systolic murmur and thrill. Ultrasonography and computed tomography revealed cystic dilatation of the superior mesenteric vein, hepatomegaly and ascites. Upper endoscopy revealed grade I esophageal varices with portal hypertensive gastropathy. The diagnosis of arteriovenous fistula between superior mesenteric artery and vein was confirmed by angiogram of the superior mesenteric vessels and resection of the fistula was performed. Control examination after nine months showed no signs of portal hypertension. Conclusion. Early diagnosis and treatment of mesenteric blood vessel arteriovenous fistula prevents portal hypertension development and its complications.