Chronic pelvic pain in a patient with Mayer-Rokitansky-Küster-Hauser syndrome: An unusual presentation

Introduction: MRKH syndrome, is a spectrum of congenital anomalies of unknown aetiology characterised by a variable degree of utero-vaginal agenesis in women with normal secondary sexual characteristics and a 46, XX karyotype. Case report: A 16 year old unmarried presented with absence of menses and chronic pelvic pain. MRKH with endometrioma was diagnosed on MRI. Laparoscopic removal of the Mullerian mass with endometriotic cyst was done. The diagnosis was subsequently confirmed on histology. Conclusion: This case highlights the atypical presentation of MRKH and reinforce the Sampsons theory of retrograde menstruation in the development of endometriosis. Patient with chronic pelvic pain and MRKH syndrome should be evaluated for endometriosis.