In patients with 46,XX DSD, serum AMH above the female range indicates the existence of functional testicular tissue suggesting the diagnosis of ovotesticular or testicular DSD, in contrast with other forms of XX virilization, such as aromatase deficiency, congenital adrenal hyperplasia of androgen-secreting tumors, which present with serum AMH in the female range (42). This evidence concerns the gene AMH and hyperinsulinemic hypoglycemia, familial, 4.