A cyanosed neonate was diagnosed as having concordant atrioventricular connection and single cardiac outlet and aorta from right ventricle with atresia of the pulmonary valve. She underwent a systemic to pulmonary artery shunt and is now thriving. Additionally, the tricuspid valve was imperforate and a large ventricular septal defect caused an unusual pattern of intracardiac blood flow. The tricuspid valve ring and right ventricular cavity appeared to be of normal size. The good outcome in this patient suggests that these features may be advantageous for corrective operation.