Introduction: Aggressive angiomyxoma is a rare soft tissue neoplasm that usually arises within perineum of woman of reproductive age. A mass in vulva, which clinically can be diagnosed as a Bertholin gland cyst, should have aggressive angiomyxoma in differential diagnosis. Rarely, cases reported in males and children. Case report: A case of 40yrs female, presented with complaints of pain in abdomen and distension of abdomen since 20 days, is being reported. On abdominal palpation: Tender, abdominal mass felt corresponding to 24-26 weeks. Per speculum examination revealed that cervix taken up. Ultrasound abdomen suggestive of 36x23x15cms neoplastic abdomino-pelvic mass, heterogenous, hypoechoic & solid. Uterus bulky with loss of endometrialmyometrial complex. Total abdominal hysterectomy with bilateral salpingoopherectomy done. Grossly, a huge 32x20x14cms tumor, weighing 2500gm, was received; whose histological diagnosis was deep aggressive angiomyxoma. Conclusion: Aggressive angiomyxoma is a rare, mesenchymal neoplasm, which infiltrates surrounding tissue. It is more common in women.
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