The laryngotracheoesophageal cleft is marked by the absence of the anatomic separation of the esophagus and the larynx. The cleft functionally can be restricted to involve the interarytenoid musculature (type 1), cricoid involvement (type 2), the proximal laryngotracheoesophagus (type 3), or missing of the whole tracheoesophageal septum (type 4). Association with significant congenital heart disease is uncommon. In this report, we are presenting a 2-week-old preterm girl presented with frequent aspiration pneumonitis, cyanosis, and heart murmur. Mechanical ventilation was extremely difficult because of endotracheal tube air leak and excessive secretions. Endoscopic evaluation revealed type 3 laryngotracheoesophageal cleft. Echocardiogram revealed double outlet right ventricle with subpulmonic ventricular septal defect. Such unique combination with severe airway disease and complex congenital heart disease was thought to constitute very high morbimortality if intervention is contemplated. It was elected to proceed with compassionate care.