Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome is a rare congenital abnormality characterised by varying degrees of aplasia or hypoplasia of the uterus and vagina. Very rarely, leiomyomas or adenomyosis can develop in the Müllerian remnant tissue or rudimentary uterus. We present a case of a 43-year-old woman with MRKH syndrome, who presented with primary amenorrhoea and lower abdominal pain. On examination, a large pelvic mass was palpated and a provisional diagnosis of ovarian tumour was made. MRI showed multiple large leiomyomas arising from the Müllerian remnant tissue, and chronic torsion of the right ovary.
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