ObjectiveTo introduce the advances in diagnosis and treatment of superior mesenteric artery syndrome (SMAS). MethodsLiteratures about SMAS published in domestic and abroad were collected and reviewed. ResultsSMAS was a rare medical condition characterized by acute or chronic ileus resulting from vascular compression of the third part of the duodenum by superior mesenteric artery. Images of upper gastrointestinal series, CT, MRI, and color Doppler ultrasonography were the major methods of diagnosing the syndrome and the upper gastrointestinal series was the most important. Conservative approaches were usually preferred to the treatment of SMAS. Surgery was performed on symptomatic patients when conservative treatment failed, and duodenojejunostomy was the best surgical procedure. ConclusionAwareness of the clinical and imaging features may be helpful to diagnosis and treatment of SMAS, and reasonable therapy shall include etiological treatment and relief of the obstruction by conservative treatment or surgery.