Acute total or subtotal occlusion of left main coronary artery (LMCA) is a catastrophic and mostly fatal event. Patients may present with cardiogenic shock and die whenever this event occurs. Survival is strongly dependent on the presence of collateral blood flow to the left coronary artery or a dominant right coronary artery, and emergency intervention for preserving the left ventricular function. Here, we present a case of a 14-year-old boy with subtotal occlusion of the LMCA accompanying acute myocardial infarction probably caused by congenital syphilis according to his positive serum syphilis antibody. His survival was closely associated with a dominant right coronary artery and timely thrombolytic therapy. Finally, he was treated with angioplasty and paclitaxel-eluting stent implantation. He was followed up after stenting and was doing quite well at the time when we wrote this paper. Acute total or subtotal occlusion of left main coronary artery (LMCA) is a catastrophic and mostly fatal event. Patients may present with cardiogenic shock and die whenever this event occurs. or a dominant right coronary artery, and emergency intervention for preserving the left ventricular function. Here, we present a case of a 14-year-old boy with subtotal occlusion of the LMCA accompanying acute myocardial infarction probably caused by congenital syphilis according to his positive His was was followed up with a dominant right coronary artery and timely thrombolytic therapy. Finally, he was treated with angioplasty and paclitaxel-eluting stent implantation. He was followed up and steved and was doing quite well at the time when we wrote this paper.