Swyer-James (Macleod) syndrome was first defined in the 1950s by Swyer, James and Macleod in patients with unilateral hyperlucent lungs. Coronary artery anomalies are congenital anomalies that affect a small part of the population. They constitute about 1-2% of congenital heart diseases. The incidence of a left coronary artery arising from the right coronary sinus Valsalva has been reported as 0.017%, and 1.3% among coronary artery anomalies. We hereby present this case since the case was diagnosed in adult age and was accompanied by a rare congenital heart disease.