A case of tracheal agenesis is reported. At present, this lethal and rare malformation cannot be recognized before birth. Circumstances of the pregnancy and the stages leading to the diagnosis at birth are reported. Autopsy allows to classify the type of malformation and to search for others malformations, as seen in the VATER syndrome. A tracheo-oesophageal fistula is always present. The mechanism is unknown and there is no chromosomal anomaly. Fifty cases have been published in literature.