Congenital atresia of the larynx is a rare, life-threatening anomaly in which early recognition and appropriate perinatal management are essential for survival. Few long-term survivors have been reported in the literature, with most documented cases studied at necropsy. The authors present a case of a full-term male newborn with Smith and Bain Type I laryngeal atresia, who has survived and developed normally during the 10-months since birth. Rapidly progressive cyanosis following umbilical cord occlusion, lack of phonation, and no air movement with respiratory efforts are typically present and should alert the clinician to the possibility of laryngeal atresia. Positive pressure ventilatory assistance may be possible through a patent pharyngoglottic duct or tracheoesophageal fistula until a surgical airway is established. If this is not possible, emergent tracheostomy may be a lifesaving procedure in the first minutes of life.