Willkie’s syndrome or superior mesenteric artery (SMA) syndrome is a rare cause of gastrointestinal obstruction, due to exterior compression of the horizontal part of the duodenum by the SMA. For the study of the clinical, diagnostic and therapeutic characteristics of Wilkie’s syndrome in the Iberian and Ibero-American population, we compiled the reports of 150 cases by Iberican and Ibero-American authors, and we found a predominance of cases in females over males in a ratio of 2.57: 1. Fifty percent of the cases were in patients under 20-years of age, with the highest number of cases in schoolchildren and adolescents. The most frequent symptoms were abdominal pain, vomiting and weight loss. The disease had two forms of presentation: acute with signs of high intestinal obstruction (16.47%), and chronic (83.53%). Diagnostic radiographic studies showed in of the upper gastrointestinal tract narrowing of the horizontal portion of the duodenum and delay in the transit of contrast through the gastroduodenal region, with or without gastroduodenal dilation in 85% of cases. In computed tomographic studies it was observed decreased mesenteric-aortic angle and distance. Upper endoscopic studies showed narrowing of the horizontal part of the duodenum, with or without gastroduodenal dilation in 61.90% of cases. Of the 144 patients that received treatment, 94 cases received medical treatment, with a success rate of 62.77%; and 84 cases required surgical treatment, 59.52% of these, upon admission. The most practiced surgical procedure was laterolateral duodenojejunostomy in 70.24% of cases. We concluded that Wilkie’s syndrome is an entity that results from the reduction of the mesenteric-aortic angle and distance, with two forms of presentation: acute and chronic, that required surgical treatment in 58.33% of cases.