Usually, StAR variants are associated to lipoid congenital adrenal hyperplasia (LCAH), in which the alteration of the StAR-dependent cholesterol import deeply impairs cortisol, aldosterone, and androgen production, both in the adrenal gland and in gonadal steroidogenesis, leading to AI and, in males, to lack of normal virilization of external genitalia [24]. Here, STAR is linked to congenital lipoid adrenal hyperplasia due to STAR deficency.