An isolated, single coronary artery is a rare congenital anomaly with an incidence of 0.03% to 0.4% and an uncommon finding (0.6% to 1.3%) in patients undergoing coronary angiography. In this report we describe a rare case of a patient who had an anomaly of the left and right coronary arteries with a single coronary ostium in the right sinus of Valsalva, in which percutaneous coronary intervention (PCI) was successfully performed in both arteries.