A 1,450-gram infant presented with respiratory distress at birth, and intubation of the airway was attempted. However, in spite of several attempts to establish an airway, the tube repeatedly entered the esophagus. Despite vigorous resuscitative efforts, the infant expired at 11 hours of age. A postmortem barium esophagram revealed that the esophagus was well distended with barium; that barium and air had entered the stomach; and that barium was present in the bronchial tree, suggesting the presence of a bronchoesophageal fistula. The findings of the otolaryngologist coupled with the results of the esophagram led to the diagnosis of tracheal agenesis. Since 1900 only about 40 cases of this abnormality have been reported. If a newborn is in severe respiratory distress, has no cry, and is difficult to intubate, tracheal agenesis should be suspected.