Abstract: Superior mesenteric artery aneurysm (SMAA) is a rare vascular disease entity with high risk of rupture and mortality. Patients are treated either with surgical intervention or conservative observation. Traditional open surgery includes simple ligation, aneurysmectomy with reconstruction of the mesenteric artery, and simultaneous bowel resection. Endovascular treatment is considered less invasive and preferred in patients with complex comorbidities or branch aneurysms. Embolization and endovascular stenting are the major choices of endovascular treatment. Given the knowledge on the management of SMAA is relatively limited, clinical decisions should be made with great caution, regarding the etiology, pathophysiology of SMAA, and the risk of perioperative and long-term complications.