Abstract
Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome involves complete or partial agenesis of the female reproductive tract, leading to impaired menstruation and reproduction, or even clinical symptoms in certain cases. The absence of an anatomically intact reproductive tract usually misleads clinicians to ignore the possibility that common gynecological conditions may develop in MRKH patients, which is a rare but plausible scenario. In the present report, we present the rare case of a 49-year-old MRKH patient with uterine fibroid developing from rudimentary uterine tissue, one of only a few similar instances reported in medical literature. The patient, scheduled to undergo oophorectomy due to a suspicious ovarian cyst and being perimenopausal, decided to undergo total laparoscopic en bloc excision of the ovaries, salpinges, uterine rudiments, fibroid, and ovarian cyst. The surgery was successful and the patient made a swift, uneventful recovery. This report adds to the limited knowledge of benign mesenchymal tumors arising from mere remnants of myometrium and affirms the need for vigilance during the care of MRKH patients.