This is the first description of treatment with coronary stenting of a patient with a single coronary artery originating from the right Sinus Valsalvae and suffering from acute inferior myocardial infarction. Angiography showed the following: the right coronary artery (RCA) had a normal course, whilst the left anterior descending (LAD) as well as the left circumflex (LCX) branches both originated separately from the proximal RCA, which served as a common mixed trunk. The LAD crossed to the left in front of the right ventricular outflow tract, whilst the LCX, taking a retroaortic course to the atrioventricular groove, had a distal occlusion. According to an anatomically based classification considering all imaginable variations, this configuration corresponds to a II-D-1 pattern, which previously has been described only in a single postmortem case. After the LCX was reopened with a guide wire, a coronary stent was successfully inserted, resulting in TIMI-3 flow. Recovery was uneventful.