Transposition of the great arteries (TGA) is a lethal condition without intervention. Cross-sectional echocardiography is the diagnostic investigation of choice. Intravenous infusion of prostaglandin is employed to maintain ductal patency and allow mixing of blood, thus improving tissue oxygenation. Balloon atrial septostomy is recommended once the diagnosis is made. The arterial switch is accepted as the best option for simple TGA. Late follow-up includes survivors of the intra-atrial repair (Mustard and Senning operations), and the emerging cohort of survivors of the arterial switch procedure. Arrhythmia, baffle stenosis, tricuspid valve dysfunction, systemic ventricular dysfunction, and sudden death may occur late during follow-up after the Mustard or Senning procedure. There are less data for late follow-up after arterial switch; however, late death is rare, usually is related to reoperation, and important arrhythmias are uncommon. The long-term fate of the coronary circulation is unknown but coronary arterial obstruction has been reported. Continuing long-term surveillance is essential to detect the development of late problems in all groups of survivors.