Large Fibroid arising from Mullerian Remnant Mimicking as Ovarian Neoplasm in a Woman with MRKH Syndrome

In this article, a large leiomyoma with degenerative changes arising from the rudimentary uterine knob in a patient with MayerRokitansky-Kuster-Hauser (MRKH) syndrome and mimicking an ovarian neoplasm is reported. The patient was a 39-year-old woman, known case of MRKH with vaginal aplasia who presented with pain abdomen, loss of appetite and weight. On physical examination, a large pelvic mass was detected. A provisional diagnosis of left ovarian neoplasm was made on USG and CT scan for which an exploratory laparotomy was performed. Finally, it was diagnosed as a case of multiple leiomyomas with hyaline degeneration on histopathological examination. Myoma arising from a rudimentary uterine knob/anlage is a rare finding but should be considered in the differential diagnosis of pelvic mass in patients with MRKH syndrome.