The celiac axis compression syndrome (CACS) due to median arcuate ligament (MAL) was first described by Harjola in 1963; originating postpandrial abdominal pain, weight loss, epigastric bruit and celiac axis stenosis > 75% in angiographic studies. This clinical condition has been the origin of controversies about its pathogenesis, diagnosis and its long term clinical results. Advances in diagnostic imaging as 64 multidetector-row CT (MDCT), 3-D reconstruction, magnetic resonance (MR) and color duplex ultrasonography, provide better understanding of the syndrome and allow to identify the best candidates for surgical division of MAL fibers. Since the introduction of laparoscopic approach, and also endovascular procedures, in 2000, a new perspective has established in this challenging syndrome. With the occasion of our own experience, a critical review of the syndrome is presented.