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BACKGROUND: Leiomyoma with secondary changes arising from Mullerian remnant may mimicovarian tumor in women with Mayer-Rockitansky-Kuster-Hauser (MRKH) syndrome in patients withpelvic mass and urologic abnormalities.CASE DETAIL: The patient was a 40 years old known case of MRKH, presented with lower abdominalpain and swelling for over one year. On physical examination, large pelvic mass (about 15cm by 10cm)was found and a diagnosis of ovarian tumor was made by imaging which also showed absent rightkidney. Finally, the mass was found to be a big leiomyoma with areas of hyaline, edematous and cysticdegenerations.CONCLUSION: Although leiomyoma arising from rudimentary Mullerian bulb is a rare condition, itshould be considered in differential diagnosis of pelvic mass in patients with MRKH. Possibility ofurologic abnormalities should also be considered in these patients and appropriate work up per localcontext should be made.