Abstract
Primary ovarian leiomyosarcoma is a very uncommon and aggressive neoplasm. We presented a right-sided ovarian leiomyosarcoma in a woman in her late 40s. No case has been described in the literature till now of primary ovarian leiomyosarcoma in a woman with uterovaginal prolapse. A total abdominal hysterectomy with bilateral adnexectomy, metastasectomy, excision of large tumour deposit over small intestine followed by resection with ileo-ileal anastomosis and omentectomy was performed. The diagnosis was made based on morphology along with immunohistochemistry. The patient was given adjuvant chemotherapy during postoperative period. Due to rarity, there is a dearth of information on the clinical behaviour and best treatment options for these tumours. This case report highlighted the importance of clinical awareness and aimed to provide a baseline to guide clinical practice as well as future research.