Origin of both great vessels from the right ventricle with intact ventricular septum is extremely rare, only two cases of this cardiac malformation has been reported previously according to our knowledge. Reported herein, is the case of a two-day-old infant who exhibited cyanosis at birth and an ejection systolic murmur along the left sternal border with a normal ECG. Necropsy observation revealed origin of both great vessels arising from a dilated right ventricle with intact ventricular septum. An atrial septal defect, hypoplasia of the mitral valve and left ventricle were present. The intracardiac hemodynamics are similar to aortic valvular atresia and/or mitral atresia with intact ventricular septum.