Abstract

Mayer–Rokitansky–Küster–Hauser (MRKH) syndrome is a rare cause of primary amenorrhea with an incidence of 1 in 5000. As per the American Society of Reproductive Medicine (ASRM) classification of Müllerian duct anomalies, uterine and vaginal agenesis correspond to class 1, the extreme form of which is the MRKH syndrome. Although considered rare, it is surprisingly the second-most common cause of primary amenorrhea after hypogonadism. The importance of the psychosocial dimension in the management of this condition cannot be overemphasized. Here, we describe a case of MRKH syndrome in a young girl from South India.

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