Superior mesenteric artery syndrome (SMAS) is characterized by compression of the third portion of the duodenum between the aorta and the superior mesenteric artery (SMA). The etiology of SMAS can be congenital or acquired, is often linked to significant weight loss, and may develop secondary to median arcuate ligament syndrome (MALS). Clinically, symptom presentation is similar to a small bowel obstruction and may include symptoms of epigastric fullness, nausea, bilious emesis, and postprandial abdominal pain. Diagnosis of SMAS requires a high index of suspicion, symptom correlation, and supporting imaging studies, with CT angiography being crucial for assessing aortomesenteric angle and distance. Surgical intervention is indicated for patients unresponsive to conservative treatment. Among several surgical options described, duodenal derotation with duodenoduodenostomy has shown promising results in alleviating symptoms. The complexity of the SMAS disease process necessitates careful diagnosis and patient selection as well as an understanding of effective surgical approaches.