A post-mortem heart with double outlet left ventricle was studied and a new type was described. An enlarged right atrium was divided by a large Eustachian valve and an atrial septal defect type ostium II, was present. The tricuspid valve was hypoplastic and the intercordal spaces were completely fused, similar to a tricuspid atresia. The right ventricle was hypoplastic. The aorta and pulmonary artery were totally related to the left ventricle with no conus beneath either. A sub-aortic ventricular septal defect secondary to malalignment of the septum was present. Subpulmonary and valvular pulmonary stenosis were observed with a hypoplastic main pulmonary artery. The left pulmonary artery was atretic and was filled by a patent ductus arteriosus. This type of double outlet left ventricle is amenable to total surgical correction if a correct prior diagnosis is made.