Single coronary arteries are rare congenital anomalies in which the whole heart circulation is supplied by a coronary artery arising from a single ostium. Single left coronary artery with right coronary artery (RCA) originating from distal left circumflex artery (LCX) is a very rare anomaly with only few cases reported in the literature. We report a 44 years old male presenting with anterior myocardial infarction who was found to have a single left coronary artery during angiography. RCA had an abnormal origin arising from distal of a dominant LCX that retrogradely followed the course of a normal RCA to the base of the heart. A brief review of the reported cases with emphasis on the clinical significance of this unusual anomaly is presented.