We experience a 1 day old female patient with congenital esophageal atresia and tracheoeso-phageal fistula combined with a subglottic laryngeal stenosis which was 2 mm in diamete. At the time of surgery, an attempt was made to intubate, but it was impossible to pass a tube beyond the vocal cord which was mormal in size and shape. Thus a gastrostomy was performed only under mask with pure oxygen and local anesthesia. The child died one day later due to respiratory failure. Congenital subglottic laryngeal stenosis combined with esophageal atresia and tracheoeso-phageal fistula is very rare, but it is a very critical anomaly to anesthesia and resuscitation. Therefore, in the case of esophageal atresia and tracheoesophageal fistula with signs of respiratory obstruction, the possible presence of subglottic laryngeal stenosis should be considered and accurately assessed.