GNRHR and hypogonadotropic hypogonadism: It encodes the GnRH receptor, comprising 3 coding exons, and contains 7 transmembrane domains, inducing LH and FSH secretion.[6] Hypogonadotropic hypogonadism-7 with or without anosmia is caused by homozygous or compound heterozygous mutations in the GNRHR gene.[7,8] Interestingly, female patients of nIHH due to biallelic GNRHR mutations have been scarcely reported, but some cases are exceptions.[9,10] Women with nIHH/bi-GNRHR showed variable puberty, but nearly all present with primary amenorrhea.[10]