Tetralogy of Fallot (TOF) is the most common cyanotic congenital heart disease. It is characterized by an interventricular communication with an overriding aorta, subpulmonary obstruction, and consequent right ventricular hypertrophy. The potential for late complications is an important concern for growing number of survivors after surgical repair, although long-term survival rates are excellent. Progressive pulmonary valve regurgitation leading to right heart failure and arrhythmias are common late complications and major reasons of mortality. In this review, we focus on research progress of pathogenesis and treatment of late complications after TOF repair, and the importance of long-term follow-up is emphasized.