The result of the study includes the data obtained by analyzing a clinical case of upper mesenteric artery syndrome, as well as statistical data and analysis of the literature in this field. CT examination with angiography is obligatory part of the overall assessment of the patient suspected with this pathology.  The superior mesenteric artery syndrome was secondary in congenital malrotation of the duodenum. The specificity of this case consists in the severity of the complication of a benign malformative disease, as well as in its surgical resolution. Clinical-paraclinical examinations, especially TC imaging with angiography allow an early diagnosis. The surgical management for upper mesenteric artery syndrome still instigates numerous discussions, especially for cases with complications, in which the tactic is established intraoperatively depending on the nature of the malformative anomaly.

Keywords: Superior mesenteric artery syndrome, Wilkie syndrome, arteriomesenteric occlusion of the duodenum, diagnosis, treatment