Superior mesenteric artery syndrome is a rare disease that should be considered in the differential diagnosis of upper abdominal pain, vomiting and weight loss. Compression of the third part of the duodenum between the superior mesenteric artery anteriorly and the aorta and the spinal cord posteriorly may result in high intestinal obstruction. Surgical treatment with duodenojejunostomy is indicated if proper conservative treatment has failed. Two patients are reported in the present case. One patient presented with acute surgical abdomen, which was an unusual presentation of superior mesenteric artery syndrome, while the second patient had classic features of the syndrome. Both patients were successfully treated with duodenojejunostomy. The pathophysiology, clinical presentation, investigation and management of this condition are discussed.