Report: central diabetes insipidus and schwannoma in a male with X-linked congenital adrenal hypoplasia.

Background: DAX1 mutations are related to the X-linked form of adrenal hypoplasia congenita (AHC) in infancy and to hypogonadotropic hypogonadism (HH) in puberty. We report a male patient affected by X-linked AHC who presented with central diabetes insipidus and schwannoma in adulthood, which has not been described in association with AHC.

Case Presentation: A 36-day-old male infant who presented with severe dehydration was admitted to the intensive care unit.