Loss-of-function of NR0B1 gene is responsible for AHC, which typically affects male children as primary adrenal insufficiency especially during the neonatal period.1,2 In addition, patients with AHC have hypogonadotropic hypogonadism leading to impaired spermatogenesis during puberty. Here, NR0B1 is linked to hypogonadotropic hypogonadism.