While AMH is low or undetectable in dysgenetic DSD, as described above, it is normal/high in steroidogenic defects because the androgen inhibitory effect on AMH is lacking and the elevation of serum FSH upregulates AMH secretion [41], particularly in the first 3–6 months after birth and at pubertal age in those cases where gonadectomy has not yet been performed (Table 1 and Figure 4). Here, AMH is linked to disorder of sexual differentiation.