Truncus arteriosus is a rare cyanotic congenital heart defect that involves septation failure of the heart’s main arterial outflow tract. Varying morphologies of the truncal valve and aorta have been reported; however, the ascending aorta is typically supplied via anterograde blood flow through the truncal valve. We present the first reported case of neonatal truncus arteriosus with the ascending aorta being supplied entirely by retrograde flow.