In the largest reported series of patients, the overall angiographical incidence of congenital coronary anomalies was 1.3%. Among these anomalies, an isolated single coronary artery is a rare, congenital coronary anomaly with an incidence of 0.044-0.23%. In this kind of anomaly, the coronary arteries arise by a single coronary ostium in the right or left sinus of Valsalva. Percutaneous coronary revascularization plays an important role in the management of acute coronary syndrome, and coronary anomalies may determine a lower success rate of this intervention. We report two cases of isolated single coronary arteries who underwent successful coronary angioplasty and stenting for acute coronary syndrome.