Esophagotrachea is the most severe form of laryngo-tracheo-esophageal cleft. This congenital anomaly is due to the anomalous differentiation of the primitive cephalic gut into trachea and esophagus. We present a case of a new born female with a common tracheoesophageal canal up to the carina. Atresia ani, a vulvo-vestibular fistula, sacral ipoplasia and others associated anomalies were also present. The baby underwent surgery after a laringo-tracheoscopy and a barium study of the esophagus. The prognosis of this extremely rare malformation is generally poor and the baby died on the fifth day after surgery for a serious ipertensive pneumothorax.