Among these, NR0B1-related adrenal hypoplasia congenita (AHC) represents a distinctive X-linked disorder characterized by combined adrenal insufficiency and hypogonadotropic hypogonadism (HH) due to loss-of-function variants in the NR0B1 gene encoding the DAX-1 protein [2,3]. The gene discussed is NR0B1; the disease is hypogonadotropic hypogonadism.