Leiomyomas in both uterine remnants in a woman with the Mayer-Rokitansky-Küster-Hauser syndrome

Objective To describe a case of Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome associated with bilateral myomas in uterine remnants. Design Case report. Setting University hospital. Patient(s) A 35-year-old woman with MRKH syndrome and pelvic pain, in whom leiomyomas were diagnosed by imaging techniques. Intervention(s) Diagnostic laparoscopy followed by excision of leiomyomas and uterine remnants by laparotomy. Main Outcome Measure(s) Resolution of symptoms and histologic confirmation of the clinic diagnosis. Result(s) After a 6-month follow-up period, the patient was asymptomatic. The histologic examination confirmed MRKH syndrome associated with multiple myomas in both rudimentary uteri. Conclusion(s) This is an unusual case of MRKH syndrome associated with bilateral leiomyomas. The excision of the tumors with the adjacent uterine remnants is indicated when they are symptomatic.