Celiac artery compression syndrome is a rare vascular disease, with incidence estimated at 0.4%. However, asymptomatic but hemodynamically significant celiac artery compression is found incidentally in 2.4–8% of the population on examination. The disease is caused by compression of the median arcuate ligament and celiac axis nerve fibers on the celiac artery, usually during expiration, and can cause symptoms of “abdominal claudication”. These symptoms include post-prandial epigastric pain, nausea, vomiting, mild weight loss and, less frequently, diarrhea, heartburn, abdominal bloating, constipation, arrhythmias and syncope. Ultrasound, computed tomography (CT), magnetic resonance (MR) and angiography are employed to diagnose celiac artery compression syndrome. Treatment of this disease is based on median arcuate ligament lysis and celiac ganglionectomy either by laparoscopic or open method, with both techniques having similar outcomes.