Abstract
The birth of a child whose examination of the genitals does not allow declaring sex is always a difficult and painful event. It involves the intervention a multidisciplinary team that will work with the family, and among them, the pediatrician which occupies an essential place at the arrival of the child. It is a neonatal emergency requiring early treatment (in case of congenital adrenal hyperplasia - CAH). Since 2005, the revised nomenclature, the new classification of disorders of sex development (DSD) and the identification of a plethora of genes required for gonad (Chicago). This consensus proposes to classify the various anomalies according to the karyotype, and eliminates the terms considered too pejorative, often used, as "sexual ambiguities", "Sexual reversion" and "pseudohermaphroditism". The unique term that has been proposed and accepted is that of DSD (Disorders of Sexual Development). The choice of the sex of civil status (possibly different from the genetic sex) depends on the data present and future hormonal data (puberty and fertility). It results from a multidisciplinary decision (geneticist, endocrino-pediatrician, surgeon, pediatrician) clearly exposed to the family.