Wilke's syndrome is a rare cause of partial or complete duodenal obstruction due to exterior compression of the third part of the duodenum by the superior mesenteric artery. It is a true syndrome with characteristic clinical picture which should be included in the differential diagnosis of chronic duodenal ileus. It is initially treated conservatively and then operatively in case of failure of the conservative treatment or if the patient is not willing to follow the medical treatment. We present a case report of Wilke's syndrome with a literature review on this pathologic entity.