Urinary retention unveiling deeply embedded multiple leiomyomas in women with Mayer-Rokitansky-Kuster-Hauser syndrome and its successful laparoscopic management: a case-report and literature review.

Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome is the second most common cause of primary amenorrhea with an incidence of 1:4000-5000 women. It is characterized by aplasia or hypoplasia of the uterus and the upper two-thirds of the vagina with normal ovaries and tubes and a normal secondary sexual characteristics. The occurrence of leiomyoma is common but it is rare to have leiomyoma in uterine remnant in MRKH syndrome. Although few cases of MRKH syndrome with leiomyoma have been reported in the literature, none presented with urinary retention. Here, we report a case of 28-year-old women who presented with urinary retention that unmasked deeply embedded huge fibroids in pelvis arising from a rudimentary uterine horns and its safe management via laparoscopic approach.