We report the case of a 49-year-old woman who complained of strong epigastric pain, emesis, diarrhea, and a weight reduction of 10 kg. The diagnosis of a coeliac axis compression syndrome was made based on a typical angiographic picture. Half a year after the operative resection of the median arcuate ligament and the coeliac plexus, the patient is completely symptom-free and her weight has increased by 4 kg. In our opinion, the most important prerequisite for the indication to operate is the careful exclusion of all other possible diagnoses in combination with all the typical findings.