Right aortic arch, a congenital malformation of the great vessels, can cause compression of trachea and/or esophagus. We experienced a case of ventilatory compromise in an infant with tetralogy of Fallot and right aortic arch immediately after insertion of transesophageal echocardiography probe during cardiac surgery. Although intraoperative transesophageal echocardiography can be safely performed in infants with congenital heart disease, it should be done with caution in patient with similar vascular malformation.