Abstract
Mayer-Rokitansky-Kuster-Hauser syndrome (MRKH) is diagnosed in one in 4000-5000 newborn girls. This syndrome is characterized by the female phenotype, normal karyotype 46, XX, congenital absence of the vagina or its upper part, cervix and uterus. The etiology of MRKH syndrome is still unknown, probably due to its heterogeneity. The article highlights and summarizes the literature on the probable etiological factors of MRKH syndrome development, the possibility of the reproductive function realization of patients with Müllerian agenesis. A rare case of leiomyoma in patients with MRKH, possibilities and diffi culties of diagnosis are described in the article. It is shown that the presence of MRKH syndrome does not exclude the possibility of fi broids of diff erent localization.