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After we review the current literature, surgical extirpation appears to be the treatment of choice due to potential complications, and laparoscopic approach is a feasibily and safe procedure for this pathology up to date.

The extrinsic compression of the third part of the duodenum as it passes through the aorto-mesenteric angle is known as the superior mesenteric artery syndrome (SMAS). This syndrome is a rare mechanical cause of upper intestinal obstruction, with a reported incidence of between 0.2% and 0.78%. Clinical manifestations of SMAS may be chronic or acute; chronic symptoms include intermittent gastric pain, fullness and occasional episodes of postprandial vomiting, while acute symptoms include incoercible vomiting, oral intolerance, mainly epigastric abdominal distension and abdominal pain. Surgery is recommended only when initial conservative treatment fails. Here, we report what appears to be the third published case of SMAS associated with hereditary motor and sensory neuropathy or Charcot Marie Tooth disease.