Congenital tracheal stenosis is an uncommon but life-threatening condition whose management is still debated. The rarity of this disease explains the lack of a standard management. Between 1986 and 1996, eight children younger than 1 year were referred to our Institution with a tracheal stenosis. The median age at operation was 3.15 months and the median weight was 4.5 kg. The diagnosis was made after an episode of respiratory distress in all but one and was confirmed by fiberoptic bronchoscopy. The median length of tracheal stenosis was 24.5 mm (Ranges: 4-30 mm). Only one patient was free from associated cardiovascular defect. Tracheal repair was performed under cardiopulmonary bypass in all. In three it was achieved by pericardial augmentation of the stenosed area, in four by resection and end to end anastomosis and in one by sliding tracheoplasty. Concomitant cardiac repair was performed in six. Two patients died after pericardial patch augmentation. In both, death was related to profound hypoxemia due to patch collapse. Two patients developed restenosis after resection and end to end anastomosis. They both had stent placement and one required reoperation and underwent a sliding tracheoplasty. At a median follow-up of 21 months (Ranges: 6-120) all the survivors are doing well and are free from respiratory symptoms. Bronchoscopic evaluation revealed in all a widely patent anastomosis without restenosis. In conclusion, tracheal stenosis in children remains a challenging lesion. Surgical technique, whether resection and end to end anastomosis or sliding tracheoplasty offer better results and should be discussed according to the length of the stenosis. Pericardial plasty should be used with caution.