Two cases of Gross E-type tracheoesophageal fistulae associated with interruption of the aortic arch in one and coarctation of the aorta in the other in early infancy were treated radically at Nagano Children's Hospital during the preceding 4 months until January 1994. The tracheoesophageal fistulae were noticed after the first stage surgery for the aortic arch anomalies, because of the remarkable abdominal distension under the intubated and ventilated condition of general anesthesia. Both the bronchial and esophageal optical fiber examinations were performed which proved useful to detect the fistulae. In each case, the transcervical division of the fistula was performed on an emergency basis. The external diameter of the fistula was 4 and 5 mm respectively. The fistulae dilated synchronously with ventilation. The early detection and surgical correction of the tracheoesophageal fistula can prevent serious complications such as DIC as seen in the first case probably caused by respiratory infection associated with the prolonged mechanical ventilation. Successful intracardiac repair were performed in both cases on the 25th and 7th day following the correction of the fistulae respectively.