A single coronary artery is a rare congenital anomaly with an incidence of 0.02-0.04%. We report on a 65-year-old male presenting with atypical chest pain and a history of hypertension and hypercholesterinemia, having diagnosed a very rare variant of a single coronary artery arising from the right sinus of Valsalva continuing as circumflex coronary artery (LCX) and thereafter as left anterior descending artery (LAD). Because the patient was asymptomatic on antiischemic medication and had a proposed relative benign course, we recommended medical treatment without coronary artery bypass surgery, and the patient has been in fine condition up to now (11 months after angiography).