Objectives Although the outcome of surgical treatment of congenital tracheal stenosis (CTS) has improved, surgical intervention for these patients, especially for those with associated congenital heart disease (CHD) remains challenging. In this report, we summarized our experience with these defects. Methods Clinical data were collected for children undergoing tracheoplasty and CHD surgery from January 2001 to March 2013. Bivariate and multiple regression analyses were used to determine the correlation between the variables. Results Forty-three patients underwent simultaneous repair of CTS and CHD. Their ages ranged from two months to nine years (mean 23.16 months) and weights from 3.5 to 46.1 kg (mean 10.71 kg). Associated CHD included pulmonary artery sling (n = 31), tetralogy of Fallot (n = 5), ventricular septal defect (n = 4), atrial septal defect (n = 4), double aortic arch (n = 2), and pulmonary atresia with ventricular septal defect (n = 1). Five patients underwent simple tracheal resection, eight patients underwent tracheal autograft, and 30 patients received slide tracheoplasty. There were seven deaths, five early and two late; the most common cause was the growth of granulation tissue. In bivariate analysis, the length of tracheal stenosis (p