Uncertainty surrounds both the timing and ideal form of early management of tetralogy of Fallot. Some centers perform early complete repair in all patients regardless of age, symptoms and morphology. Others recommend a two-stage approach involving initial palliation in symptomatic neonates and young infants and those with unfavorable anatomy (anomalous coronary anatomy or hypoplastic pulmonary arteries). Advantages of early anatomic correction include alleviation of cyanosis, normal growth and organ development, removal of stimulus for right ventricular hypertrophy and avoidance of risks associated with initial palliation. With recent advances in anesthetic, operative and postoperative management, routine primary repair of tetralogy of Fallot in the neonate and young infant can be accomplished with excellent early and mid-term results. However, long-term follow-up is necessary to assess the impact of early repair on late right ventricular function, arrhythmias and need for reintervention.