Anomalies of the coronary artery are incidentally detected during coronary angiography and are seldom found in daily clinical practice. In the reported studies, the incidence ranges from 0.6 to 1.3%, and men are more frequently affected. Among the different types, the detection of a double left anterior descending coronary artery emerging from the right coronary sinus is extremely rare. We describe a case of a 64-year-old male patient admitted to our institution because of ST segment elevation myocardial infarction. After successful systemic lysis, he underwent coronary angiography that showed multivessel coronary disease, a patent infarct-related artery and a double left anterior descending artery. We discuss the clinical relevance of a coronary artery emerging from the opposite Valsalva sinus and its clinical implications.