Although mutations in steroidogenic acute regulator protein (STAR) have classically been associated with congenital lipoid adrenal hyperplasia, characterized by severe deficiency of both glucocorticoids and mineralocorticoids and no androgenization of 46,XY fetuses (13), partial loss-of-function variants in STAR are increasingly reported in patients diagnosed with FGD who presented with later onset, milder disease, and apparently normal gonadal function (14–16). This evidence concerns the gene STAR and Aarskog-Scott syndrome, X-linked.