Background: Superior mesenteric artery syndrome is uncommon and characterized by postprandial epigastric pain, nausea, vomiting, anorexia and weight loss. The syndrome is caused by compression of the third part of the duodenum in the angle between the aorta and the superior mesenteric artery. This review updates etiology, epidemiology, diagnosis, treatment and outcome of the superior mesenteric artery syndrome. Methods: Review of the literature. Results: Frequently, predisposing medical conditions associated with catabolic states or rapid weight loss result in a decrease of the aortomesenteric angle and subsequent duodenal obstruction. External cast compression, anatomic variants and surgical alteration of the anatomy following spine surgery or ileoanal pouch anastomosis can also precipitate the syndrome. Once radiologic studies have established diagnosis, first-line treatment is usually conservative with jejunal or parenteral nutrition for restoration of the aortomesenteric fatty tissue. If conservative management fails, surgical options include open or laparoscopic duodenojejunostomy or duodenal mobilization and division of the ligament of Treitz. Conclusion: Superior mesenteric artery syndrome is clearly defined and frequently associated with a wide range of predisposing conditions and surgical procedures; clinicians have to consider this syndrome in such a setting. Larger studies are needed to better define the optimal treatment for this disease.