Objective To investigate the surgical strategies for the treatment of tetralogy of Fallot (TOF) with left pulmonary artery hypoplasia. Methods A total of 17 TOF pediatric patients with left pulmonary artery hypoplasia were retrospectively analyzed. Among them, there were 12 males and 5 females with ages of 2 months-3 years and body weights of 4-13 kg. Curative repair or palliative repair was conducted according to the development of pulmonary artery. Regular postoperative examinations were performed, including X-ray, electrocardiogram, color Doppler echocardiography, and CT, to observe possible residual shunts, residual obstruction, and cardiac dysfunction. Results Among 17 TOF pediatric patients with the left pulmonary artery hypoplasia, 8 of them underwent patch cavoplasty of left pulmonary artery and curative repair and 7 patients survived. The 6 survived patients were followed up for 5-29 (14±10) months and among them, openings of left pulmonary artery of 2 patients were small and no complications were found among other 5 patients. One patient could not be treated due to pulmonary vein hypoplasia and died of severe low cardiac output syndrome. Among another 9 patients, 2 of them underwent single ventricular repair because of coronary artery abnormality and tricuspid valve hypoplasia, respectively, and 7 of them underwent palliative repair. Among 7 patients undergoing palliative repair, 5 of them underwent patch cavoplasty of left pulmonary artery; 6 of them survived, 5 of them were followed up for 6-18 (17±13) months and among them, there were 4 patients with left pulmonary hypoplasia and 1 patient died of cardiac dysfunction and multiple organ function failure. Conclusion TOF with left pulmonary artery hypoplasia should undergo the curative repair as far as possible based on patch cavoplasty of left pulmonary artery.If it is possible to develop the residual stenosis of left pulmonary artery after repair, the surgeon can punch a hole in the ventricular septal defect patch.