Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome, often viewed as the female counterpart of Zinner syndrome, has been associated with LIM homeobox 1 (LHX1), hepatocyte nuclear factor 1 Beta (HNF1B), and Wnt family member 4 (WNT4) mutations in prior studies, which are critical for the development of the Wolffian duct [20]. Here, LHX1 is linked to Mayer-Rokitansky-Kuster-Hauser syndrome type 1.