Mesenteric ischemia is an uncommon etiology of abdominal pain. Celiac axis compression syndrome is an extremely rare cause of mesenteric ischemia. The primary pathological mechanism is the external compression of the celiac trunk by median arcuate ligament. The clinical manifestation of celiac axis compression syndrome includes postprandial pain, diarrhea, and body weight loss. The diagnosis of this disease is usually difficult and depends on the angiography findings. For treatment, only percutaneous transluminal angioplasty and surgical intervention have been suggested in reviews in the literature. We, herein, report an unusual case of celiac axis compression syndrome and also review the literature pertaining to this disease.