Congenital esophago-bronchial fistula in early childhood is exremely rare. The clinical details of a 22-month-old boy with esophago-bronchial fistula are reported. He was referred to our hospital for recurrent pneumonia and abdominal distention. Digital subtraction esophago graphy revealed a fistula between the mid-point of the esophagus and the left main bronchus. Bronchoscopy showed left bronchomalacia and an opening in the fistula at the membraneous portion of the left main bronchus. Surgery was performed through a right extrapleural thoracotomy at the 5th costal floor. After excision of a short wide fistulous tract resembling a cleft, the esophagus and left main bronchus were repaired with 4/0 vic-ryl sutures and 5/0 nylon sutures, respectively. Stenosis of the left main bronchus developed after the operation and was identified by bronchofiberscopy. The stenosis was successfully enlarged by balloon catheter dilatation. The patient is now doing well.