Agenesis of the trachea is a rare anomaly. The main signs are respiratory distress and cyanosis, inability to vocalize and impossible tracheal intubation. In most cases concomitant congenital anomalies of the heart, digestive tract or genitourinary tract are present. Endoscopy and X ray studies will confirm the diagnosis. There is no long-term surgical solution because no suitable material for a tracheal prosthesis is available at present, therefore the condition is ultimately fatal. We report a case of tracheal agenesis. After the diagnosis was established the baby's lungs were ventilated for several hours via an oesophageal tube and two broncho-oesophageal fistulae, but she finally died from untreatable respiratory acidosis. Autopsy revealed a Floyd's type III tracheal agenesis and a laryngeal cleft.