Congenital left main coronary atresia (LMCA) is a rare coronary anomaly associated with myocardial ischemia and sudden death. Echocardiography is an important tool for diagnosis of LMCA. The results of echocardiography and relevant clinical signs were retrospectively analyzed in 3 children with LMCA. Echocardiographic examination showed the signs of LMCA: in left coronary sinus no opening of left coronary artery (LCA), retrograded blood flow in LCA, abound blood flow in ventricular wall, and mild widen RCA.