An isolated single coronary artery is a rare congenital anomaly with an incidence of 0.03% to 0.4% and can present additional hazards such as myocardial ischemia, infarction, or sudden death in individuals with atherosclerotic changes in this artery. Herein, we report an acute anterior and inferior wall infarction in a 61-year-old male patient with the right coronary artery arising as a branch of the left coronary artery. Selected coronary angiography showed a 90% stenosis in the left anterior descending artery proximal to the origin of the right coronary artery. The patient underwent successful percutaneous transluminal coronary angioplasty and stenting without compromising the flow to the anomalous right coronary artery. There has been no previous reports of percutaneous transluminal coronary angioplasty and coronary stenting in a patient with this anomaly.