When replacement of the aortic valve in neonates and infants is required, the Ross-Konno procedure provides a high-quality left ventricular outflow with good mid-term durability. The procedural outcome is highly dependent on the pre-procedural condition of the neonate or infant. Most series contain substantial numbers of post-balloon valvotomy patients as emergent or urgent management of severe, acute regurgitation. In patients with compromised ventricular function, post-operative mechanical support may be required but is generally associated with a good outcome. The neonatal and infant autograft may perform better in the long term than un-supported autografts performed later in life. Early definitive or delayed treatment of left ventricular outflow tract pathology associated with aortic interruption increasingly involves an infant Ross-Konno. The procedure also has a role in the recruitment of small left ventricles. Concomitant mitral pathology or aortic obstruction magnifies complexity, but the operative approach to the Ross-Konno remains the same.