Newborns who present with DSDs must be considered medical emergencies due to the life-threatening issues that present for some cases and the social implications in all cases. A work-up should be started immediately in the attempt to obtain a precise diagnosis as early as possible. In 46,XX DSD, female sex of rearing is preferred as most cases will develop the potential for fertility despite masculinization of the external genitalia. Additionally, much evidence indicates that development of female gender identity is typical in those cases. In 46,XY DSD, the appearance of the genitalia at birth plays a stronger role in decisions regarding sex assignment. Female sex of rearing is preferred in complete DSDs, particularly CAIS or Swyer syndrome. Male sex of rearing is recommended for cases of congenital micropenis and 5α-reductase deficiency. Following a proper diagnosis, parents must be educated about DSDs including what is known about long-term outcome. Following this educations, the medical team should support the parents as they decide on an appropriate sex of rearing for their affected newborn.