Interrupted aortic arch with aortic atresia is an unusual complex of congenital heart disease. Incompatibility for life is a potential risk unless there is blood flow in the ascending aorta supplying the coronary arteries from another existing malformations. We report a five days old case with interrupted aortic arch type B, aortic atresia, ventricular septal defect and an aortopulmonary window as a source of blood flow to the coronary arteries, ascending aorta and proximal aortic arch. A successful surgical correction with biventricular repair was performed. The significance of this is to expand the candidates for one stage total correction of congenital heart diseases in neonates, which spares patients multiple staged surgeries and spares health service providers the burden of multiple treatment strategies.