Objective: To compare the efficacy of total thoracoscopic minimally invasive cardiac surgery and conventional open heart surgery in the treatment of congenital heart disease. Methods: A total of 67 cases of congenital heart disease received from April 2013 to December 2016 were randomly divided into the observation group (34 cases) which with three holes minimally invasive surgery and the control group (33 cases) which with traditional thoracotomy surgery. One patient in the observation group was converted to conventional open chest surgery for complex congenital heart disease. The two groups were compared in patients with cardiopulmonary bypass time, aortic clamping time, operation time, postoperative ventilation time, 24 hours postoperative of thoracic drainage, postoperative ICU time, postoperative hospitalization time and complication rate. The life quality one week after the operation of the two groups was also compared with the Chinese version of the European Organization for Research on Treatment of Cancer (EORTC) life quality scale QLQ-C30(V3.0). Results: The patients in the observation group were better in postoperative ventilation time, 24 hours postoperative of thoracic drainage, postoperative ICU time, postoperative hospitalization time and the life quality one week after the operation（P<0.05）, and worse in the cardiopulmonary bypass time and aortic clamping time than those in the control group（P<0.05）. There was no significant difference in the total operation time and the incidence of complications between the two groups. All the patients recovered smoothly without serious complications or death, and had no long-term death or atrioventricular recanalization after 3-24 months of follow-up. Conclusion: Total thoracoscopic minimally invasive cardiac surgery is safe and effective for simple congenital heart disease, and has the advantages of less destruction, less trauma, less bleeding, faster recovery, high early postoperative life quality and less impact on the appearance than traditional thoracotomy surgery.