Abstract Congenital anomaly of the coronary arteries is a potentially lethal disease, especially in young people. Currently, non-invasive cardiovascular imaging tests have shown better definition of the origin and course of the coronary arteries. Surgical treatment should be indicated in symptomatic patients, while the ideal management of asymptomatic patients remains controversial. We report a case of anomalous origin of the left coronary artery from the right coronary sinus with an interarterial course, in an asymptomatic patient treated with revascularization surgery.