Wilkie's syndrome is a rare disease that should be considered in the differential diagnosis of upper abdominal discomfort and weight loss. Compression of the inferior part of the horizontal duodenum by the superior mesenteric artery may result in high intestinal obstruction with postprandial or positional discomfort, vomiting and weight loss. The diagnosis is based on clinical presentation and confirmed by radiographic studies during a symptomatic period. Therapy of first choice is conservative. Nevertheless, surgical intervention with duodenojejunostomy is often required.