BACKGROUND Aortotracheal fistula (ATF) is an uncommon and fatal complication of tracheal or aortic surgery, especially among pediatric patients. METHODS We reported a case in a 1-year-old boy with dextrocardia, left pulmonary artery sling and long segment tracheal stenosis. He received slide tracheoplasty at 9 months of age and had post-operative refractory granulation at distal trachea status post repeated balloon dilatation and laser vaporization. Episodes of hemoptysis occurred on post-operative day 81. Bronchoscopy revealed a pulsating pseudoaneurysm at lower trachea which ruptured during the procedure Urgent surgical repair under cardiopulmonary bypass with deep hypothermic circulatory arrest was done. No recurrent bleeding or significant neurologic deficits noticed at a 4-month follow-up. CONCLUSIONS Congenital anomaly that changes the spatial relationship between trachea and aorta could have contributed to formation of ATF. This warrant future attention when managing tracheal granulation with this not uncommon anatomy.