The case of a 60-year-old patient who was repeatedly admitted for ascitic decompensation secondary to hyperflow portal hypertension provoked by congenital arteriovenous malformation of the superior mesenteric artery is presented. Diagnosis was performed by arteriography. Surgical treatment was conducted with total resection of the malformation thereby achieving complete resolution and normalization of portal pressure. The literature is reviewed and the pathophysiologic mechanism as well as the clinical manifestations, diagnosis and treatment are discussed.