Transposition of great arteries (TGA) comprises 5–7% of all CHDs. It is characterized by atrioventricular concordance and ventriculoarterial discordance, resulting in the systemic and pulmonary circulations as parallel instead of the normal in-series circulation. Survival of the baby depends on mixing of blood between these two circulations either with an atrial septal defect, ventricular septal defect, or at the great arterial level via patent ductus arteriosus. Therefore, the clinical manifestation is highly variable and influenced by the presence or absence of these associated anomalies. Patients with TGA without mixing of blood present with cyanosis and acidosis and are hemodynamically compromised soon after birth and require resuscitation to re-establish connection between parallel circuits by reopening the ductus with intravenous prostaglandin (0.05–0.1 μg/kg/min) or establishing interatrial flow with balloon atrial septostomy. In addition, patients may require inotropic support, ventilator support, or extracorporeal membrane oxygenation in extreme cases with refractory cardiorespiratory decompensation for survival or as a bridge to definitive therapy. TGA is uniformly fatal in the infant period, with 30% mortality in the first week of life, and 50% within the first month, and 90% in the first year of life if untreated. Fortunately, modern medical and surgical management techniques have resulted in 90% of patients living into adulthood, typically with a vigorous quality of life. Currently, the definitive corrective surgery is the arterial switch operation (ASO), as a single-stage procedure with excellent short-term and long-term outcomes. The overall perioperative survival following ASO is more than 90%. Long-term and arrhythmia-free survival is ∼97% at 25 years. All standard general anesthetics can be used safely for perioperative management, and mortality owing to anesthetic management has not been witnessed. This systematic review describes the definition and etiology of TGA, clinical presentation, pathophysiology, brief current surgical approaches, anesthetic and cardiopulmonary bypass management, and postoperative course of a patient with TGA undergoing ASO.