Within the group of congenital cardiac anomalies manifesting atrioventricular discordance, there exists a subset of hearts characterized by the additional presence of double outlet of the morphological right ventricle. Most of these hearts have associated pulmonary stenosis and abnormal direction of the cardiac apex, and all have a ventricular septal defect. Recently, a 10-year old boy underwent successful surgical correction of double outlet right ventricle with atrioventricular discordance. Diagnostic confirmation was made by 2-D echocardiography, cardiac catheterization and cineangiocardiography. The operation consisted of patch closure of ventricular septal defect so as to leave both great arteries draining the systemic[morphologically right] ventricle, and insertion of an extracardiac valved conduit between the morphological left ventricle and the distal end of the proximally oversewn pulmonary artery. Postoperative course was uneventful.