Abstract： Objective To explore the optimal approach of repairing tetralogy of Fallot with unilateral hypoplasia of pulmonary artery (UHPA-TOF).Methods A retrospective review was conducted for 51 consecutive UHPA-TOF patients from January 2006 to December 2013.The clinical data were analyzed with software SPSS 19.0.Results Thirty-five patients underwent primary repair.Two patients died with a hospital mortality rate of 6％.CICU stay length was 7 ± 6 days,hospital stay duration 21 ± 13 days and severe restenosis rate 8％.Among 16 patients undergoing palliative repair,five patients died with a hospital mortality rate of 31％.CICU stay length was 13 ± 12 days,hospital stay duration 25 ± 17 days and severe restenosis rate 71％.Hypoplasia pulmonary artery (HPA) developed better in primary than palliative repairing (P＜0.05),And patch enlargement was superior to patch non-enlargement (P＜ 0.05).Conclusions Excellent outcomes of UHPA-TOF patients may be achieved by primary repair with patch enlargement of HPA.Restenosis of HPA is a major long-term complication and it should be intervened promptly and followed up closely.