Congenital tracheo-esophageal fistula without atresia of the esophagus is a rare anomaly. Twenty-six patients are reported in the literature. The authors report an additional case. The etiology is still obscure, but may be due to the development of aberrant blood vessels. Patients present a triad of signs and symptoms, coughing and cyanosis with feedings, abnormal distention of gastro-intestinal tract and persistent pneumonitis. Radiographic investigation is most important. Lipiodol study of the esophagus in the oblique or prone position is the most rewarding diagnostic test. Surgery is the only method of cure.