The incidence of congenital coronary artery anomalies is estimated to range between 1% and 2% in the general population. The separate types of coronary artery anomalies are even rarer - the left main coronary artery arising from the right coronary sinus and passing between the thoracic aorta and the pulmonary artery is one of them. In this case, the segment of the artery that courses between the aorta and the pulmonary artery is prone to compression, especially during heavy exercise. Outcomes may be fatal due to myocardial hypoperfusion, which is associated with sudden cardiac death especially among children, young adults, and athletes. Nowadays, innate coronary artery anomalies may be incidentally diagnosed in older age using new investigation methods such as computed tomography angiography.