Based on the recognition of the cause of abnormal sex organ development in patients bearing a Y chromosome (Table 1), disorders of sex development (DSD) may be divided into (a) malformative DSD, where abnormal morphogenesis of the genital primordia occurs in early embryonic life; (b) dysgenetic DSD, due to abnormal gonadal differentiation resulting in insufficient secretion of androgens and AMH; and (c) nondysgenetic DSD, in which the abnormal sex hormone-dependent genital differentiation results from specific defects in the production or action of androgens or AMH. Here, AMH is linked to disorder of sexual differentiation.