Spontaneous isolated dissection of the superior mesenteric artery (SIDSMA) is a rare but fatal condition. Herein, we report the therapeutic outcome of a contemporary series of 12 patients with SIDSMA who were treated with conservative, anticoagulation, or endovascular therapy. Revascularization was measured according to recanalization of the primary arterial occlusive lesion and reperfusion was measured by flow through the occluded vessel. Pain was evaluated by using the visual analog scale (VAS) at admission and at each follow-up visit. Type I SIDSMA was seen in 3 (25%) patients, type IIa in 4 (33.3%) patients, and type IIb in 5 (41.7%) patients. No patient had type III SIDSMA. The false lumens were patent in 6 (50%) patients. Partial thrombosis in the false lumen was demonstrated in CT scans in 5 (41.7%) patients and total thrombosis in 1 (8.3%) patient. Four (33.3%) patients received conservative therapy, and 2 (16.7%) patients received anticoagulation therapy. All six patients resumed normal blood flow in the SMA. The remaining six patients received endovascular stenting. After stent placement, excellent distal blood flow was restored. Abdominal pain was completely resolved in all patients except in one patient. No complications associated with SMA dissection occurred. If bowel perfusion is not compromised and the SMA aneurysm is not likely to rupture in patients with a symptomatic SIDSMA, conservative, or anticoagulation therapy can be considered. If patient has sustained intestinal ischemic symptoms, and severe compression of the true lumen, or dissecting aneurysm likely to rupture, endovascular therapy, or surgery should be adopted.