We evaluated the use of duodenal derotation as a surgical option for superior mesenteric artery syndrome (SMAS) in two groups of young patients. Sixteen patients with SMAS diagnosed by barium upper gastrointestinal series (UGI) from 1974 to 2001, and six patients diagnosed by computerized tomography with three-dimensional reconstructions (3D CT) from 2001 to 2007 were referred to our surgical service, 19 of whom underwent duodenal derotation as the primary surgical treatment after a failed trial of conservative treatment. The main measured outcomes were the resolution of typical symptoms of SMAS and the development of long-term surgical complications. Of the first 16 patients, three (19%) responded to nasojejunal feedings. Of 13 patients undergoing derotation, only one (7.7%) failed derotation and required a gastrojejunostomy bypass, whereas 12 (92%) became asymptomatic after the derotation procedure. After a mean follow-up of 5.13 years (range 0.1-15), two patients (15%) presented with small bowel obstructions and were treated with a simple lysis of the adhesion. All six patients from 2001 to 2007 responded well to surgical derotation. Overall, duodenal derotations successfully relieved symptoms in 18 out of 19 (95%) patients with SMAS, with two (11%) major long-term surgical complications. No volvulus was observed in our patients at the mean follow-up of 4.37 years.