A 63-year-old mother of two, presented with blood-stained vaginal discharge and right sided lower abdominal pain. A MRI examination confirmed a right parametrial mass, abutting the lateral margin of the uterus and the patient had a total abdominal hysterectomy and bilateral salpingo-oophorectomy. Histological examination diagnosed a cotyledonoid leiomyoma, but with a new epithelioid variant. Cotyledonoid leiomyom’s usually have a large, fungating appearance and demonstrate apparent widespread infiltrative growth and extension into the pelvic cavity, broad ligament and retroperitoneal space which may raise significant concern about the possibility of a malignant neoplasm. As these tumours are rare and infrequently encountered, it is imperative that clinicians be aware of this entity as they may pose a significant diagnostic and management challenge when encountered. Awareness of this newly described epithelioid cell variant of cotyledonoid dissecting leiomyoma is necessary for an accurate diagnosis and to facilitate appropriate management decisions at the time of surgery. This new variant further emphasizes the need for meticulous histopathological assessment which should be undertaken to circumvent misdiagnosis. This has direct clinical relevance to all operating gynaecologists and may have implications for litigation because patients may be inappropriately and inadvertently over-treated for an essentially benign condition.