Congenital tracheal stenosis (CTS), though rare, is important because the mortality and morbidity rates are high in infants. Especially, associated congenital heart disease (CHD) in these infants may compound the effects of airway pathology. A 3-week-old patient with long-segmental tracheal stenosis below an anomalous right-upper lobe (RUL) bronchus had undergone a total correction of double outlet right ventricle. On third postoperative day, hypercarbia developed, and severe airway obstruction and atelectasis were detected. An emergency slide tracheoplasty was performed under cardiopulmonary bypass (CPB). The patient recovered well after the surgery. Thus, special attention needs to be paid during the postoperative intensive care of patients with congenital tracheal anomalies. Early detection and prompt diagnosis of airway obstruction can help reduce the morbidity and mortality rates. Further, it is important to select the suitable treatment of CTS associated with CHD.