We describe a 71-year-old man with a history of a small posterior myocardial infarction. Angiographically he showed a coronary artery disease with major proximal stenosis of both branches of the left coronary artery and an occlusion of the marginal branch of the circumflex artery (infarct-related vessel). As an unexpected finding, an anomalous origin of the right coronary artery from the pulmonary artery was also found. This rare condition may cause development of ischemia with potentially lethal complications as a result of coronary steal phenomenon. Surgical treatment is, therefore, indicated even in asymptomatic patients with this anomaly. Possible surgical procedures and there indications are discussed.