A 54-year-old man suffering from effort angina pectoris had an anomalous origin of the left anterior descending coronary artery (LAD) from the right sinus of Valsalva (RSV). The anomalous LAD with a small ostium and without other significant narrowing initially ran into the interventricular septum and subsequently the anterior interventricular groove. Coronary angiography during the anginal attack induced by ergometer exercise testing revealed neither an enhanced narrowing of the LAD ostium, nor myocardial squeezing of the LAD at the interventricular septum level, nor coronary vasospasm. Myocardial ischaemia associated with an anomalous aortic origin of the LAD from the RSV is extremely rare, especially when the vessel runs a septal course. In the present case, the ostial stenosis of the anomalous LAD, probably due to developed atherosclerosis, seemed to be the most likely cause of the exertional angina.