Endometrial stromal sarcoma is a hormone-sensitive, rare malignant tumour that makes around 0.2% of all uterine malignancies and is considered to occur more often in pre-menopausal women. A proper pre-operative diagnosis is difficult and confirmed in most cases after hysterectomy for a presumed benign disease. Endometrial sampling, ultrasound and MRI can provide diagnostic clues. Total hysterectomy with bilateral salphingo oophorectomy is the main line of management. Adjuvant hormonal therapy, radiotherapy and surgical excision of metastasis are recommended for recurrences. This patient, a 46-year-old parous lady presented with abdominal pain, was evaluated; CT scan showed suspected malignant left ovarian/uterine neoplasm with pulmonary metastasis. Total abdominal hysterectomy and bilateral salpingo oophorectomy was done, histopathology report showed low-grade endometrial stromal sarcoma, and immunohistochemistry showed focal CD 10 positivity. The patient responded well to Letrozole and is in remission.
