Mayer-Rokitansky-Küster-Hauser Syndrome Associated with an Ectopic Ovarian Fibroma and Solitary Pelvic Kidney: A Rare Case

Abstract

Background: Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome is a rare congenital Müllerian duct anomaly characterised by uterovaginal agenesis in women with normal ovarian function, secondary sexual characteristics, and a 46, XX karyotype. Although renal anomalies are common, ovarian tumours are exceedingly rare. Case Presentation: We report the case of a 50-year-old woman with a known diagnosis of MRKH syndrome who presented for a routine health evaluation. Ultrasonography and magnetic resonance imaging (MRI) demonstrated complete uterine agenesis, a solitary ectopic pelvic kidney, and a well-defined solid mass arising from an ectopically located right ovary, with imaging features suggestive of an ovarian fibroma/thecoma. The patient underwent successful surgical excision, and the postoperative course was uneventful, with no evidence of recurrence on follow-up. Conclusion: This case highlights the diagnostic value of MRI in accurately delineating Müllerian anomalies, associated renal abnormalities, and ectopic ovarian tumours. Reporting this exceptionally rare association expands the existing literature and emphasises the importance of comprehensive evaluation of adnexal masses in patients with MRKH syndrome.