During the last few years, our knowledge of Pierre Robin syndrome has benefited from advances in embryonic neurobiology. It has been shown that the syndrome is related to an anomalous development of the foetal brainstem. A multidisciplinary study makes it possible to value prospectively the various anomalies with which it may be associated and to decide on a coherent treatment. This type of management should reduce the mortality and prevent the sequelae of Pierre Robin syndrome.