Currently, a considerable number of genes have been linked to 46, XY DSD, such as, SRY, TSPYL1, WNT4, WT1, WWOX, ZFPM2, AKR1C2, AKR1C4, AMH, CYB5A, CYP11A1, CYP17A1, HSD17B3, HSD3B2, LHCGR, POR, and AR [7]. The gene discussed is LHCGR; the disease is disorder of sexual differentiation.