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- Genotype is XX as should be female
- Ovaries develop normally
- Foetal Adrenal Gland is hyperactive and secretes large quantities of Adrenal Androgens, due to a 21 hydroxylase mutation.
- Wolffian Ducts persist due to the presence of Androgens, thus allowing development of male internal and external genitalia.
- Mullarian Ducts persist due to the absence of testes, hence absence of Sertoli cell MIH secretion. This allows formation of female internal genitalia.
- Appears male with a micropenis and scrotum
- Both male and female internal genitalia