Congenital heart disease (CHD) is the most common type of congenital malformations. Congenital supravalvular aortic stenosis (SAS), one kind of CHDs, is strongly associated with abnormal aortic sac development with low prevalence while shows considerable impacts on infants' physical and mental development. Presently, surgical treatment is an effective therapy for SAS with acceptable surgical risk and good late survival. Evaluating the stenosis grade preoperative, the real-time status of heart during surgical correction are extremely crucial. Transesophageal echocardiography (TEE), has the advantages of clear images without interference of surgical field, is widely applied in diagnosis of cardiovascular diseases and intraoperative continuously monitoring. Here, we presented a successful case of surgical resolution of obstruction of congenital SAS in an infant who suffered difficulties in removing cardiopulmonary bypass (CPB) under the guidance of TEE.