- Etiology: 46XX disorder of sexual development, defect in adrenal steroidogenesis, androgen excess in utero leads to masculinization, most common cause is 21-hydroxylase deficiency
- Imaging: diffusely enlarged adrenal glands which are cerebriform in appearance
- Complications: potential medical emergency as salt-wasting type can be fatal
- Clinical: virilized female genitalia, female gender assignment, salt wasting in male