Double-outlet right ventricle includes a variety of anatomic arrangements wherein both great arteries, aorta and pulmonary artery, associate primarily with the right ventricle. It has a variety of anatomic subtypes depending on the relationship of the ventricular septal defect to the great arteries and the presence or absence of other coexisting cardiac anomalies. The subtypes of double outlet right ventricle are examined in detail along with their physiologic implications. This chapter then discusses in detail the physiology, clinical presentation. and perioperative management of a child with double-outlet right ventricle, a ventricular septal defect, and associated pulmonary stenosis.