External compression and kinking of the celiac axis is caused by the enlarged and fibrous median arcuate ligament of the diaphragm. The pathogenetic cause may be a functional ischemia or an irritation of the squeezed celiac ganglion. This painful syndrome is an unusual condition, which occurs at a maximum age of about 40 years and mostly in women. The diagnosis is established by exclusion of all other abdominal sources of pain and lateral aortography. The therapy can often be limited to longitudinal incision of the ligament; reconstructive procedures of the coeliac artery are sometimes necessary.