A 25-month-old patient with complete atrioventricular septal defect and Tetralogy of Fallot underwent repair of both anomalies. The diagnosis was established preoperatively by 2D-echocardiography, cardiac catheterization and cardioangiogram, Repair was accomplished using cardiopulmonary bypass and profound hypothermia to 18C, Closing of the atrioventricular septal defect was achieved with the use of two Dacron patchs by an atrial approach alone. Infundibulectomy and outflow tract reconstruction with the transannular pericadial patch containing a monocusp were performed. Upon the postoperative evaluation by 2D-echocardiography, mitral regurgitation was absent, but a tiny dehiscence of ventricular patch and minimal tricuspid regurgitation were noticed.