A 6-month-old infant with anomalous left coronary artery underwent a direct left subclavian-left coronary artery anastomosis without the use of cardiopulmonary bypass. Subsequently, mitral valve replacement was performed for severe mitral insufficiency diagnosed echocardiographically to be due to isolated papillary muscle infarction. To our knowledge, this is the youngest patient to have undergone both procedures during the first year of life with excellent results on follow-up studies. The case demonstrates the advantages of using direct subclavian-coronary artery anastomosis and 2-dimensional echocardiography in the management of patients with anomalous left coronary artery.