Celiac artery syndrome exists, although it remains controversial, and in some patients a firm diagnosis cannot be established. Duplex scanning or computed tomographic, magnetic resonance, or contrast aortography will confirm intermittent or permanent compression of the celiac artery by the crus of the diaphragm, the median arcuate ligament, or fibrous ganglionic tissue. Preoperative ganglion block and exercise gastric tonometry are useful diagnostic tools to predict better outcome after treatment. In patients with well-defined syndrome of chronic mesenteric and gastric ischemia or with exercise-induced pain, good results can be expected with division of the median arcuate ligament with open celiac artery reconstruction. Patients with atypical pain or history of psychiatric disorders only occasionally benefit from surgical repair. The role of primary stenting of celiac artery compression is still not well defined, and current data do not support the use of balloon-expandable stents. Laparoscopic division of the median arcuate ligament followed by celiac artery stenting is an effective, minimally invasive technique to manage selected patients with celiac artery compression syndrome.