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Mayer-Rokitansky (MRKH) Syndrome is a congenital reproductive-tract disorder in females marked by an absent or rudimentary uterus and upper vagina despite normal ovaries and normal female chromosomes.

Mayer-Rokitansky-Küster-Hauser syndrome arises from abnormal development of the Müllerian (paramesonephric) ducts during embryonic life, which normally give rise to the uterus, cervix and upper vagina. As a result, affected individuals — who are chromosomally and hormonally female (46,XX karyotype, functioning ovaries) — are born with an absent or underdeveloped uterus and the upper portion of the vagina, though the ovaries themselves develop and function normally, so puberty, breast development and other secondary sexual characteristics generally proceed normally. Because the uterus is missing or non-functional, affected women typically do not menstruate (primary amenorrhoea) and cannot carry a pregnancy naturally, though they may still be able to have biological children via assisted reproduction (their own eggs, a surrogate ut …