RESUMO
A 17-year-old girl presented with alopecia involving lateral margins of the scalp with primary amenorrhea. There was no history of parental consanguinity, and no other siblings were having similar complaints. Her secondary sexual characters were well developed with hypoplastic vagina. Histopathological findings from scalp biopsy showed features of alopecia areata. Ultrasonography of abdomen and pelvis revealed the absence of uterus and the right kidney. Follicle-stimulating hormone, luteinizing hormone, estradiol, testosterone, and thyroid function test was within normal limits. The patient had normal 46, XX Karyotype. Till date, only four case reports of Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome with alopecia has been reported. We are reporting the first case of MRKH syndrome with alopecia with normal gonadal function in world's literature.