ABSTRACT
Aims and Background: Aggressive angiomyxoma is rare tumor of pelvic and perineal organs, occurring usually in women of reproductive age, and carrying a high tendency to local infiltration and relapse. Most literature about Aggressive angiomyxomaconsists of isolated case reports. Presentation of Case: We reported a case of 41-year-old Libyan woman complaining of a slow and progressive growth of a right vulvar labia major a pedunculated tumor with long a stalk was detected, measuring of 12.0x8.0 cm. Wide surgical resection of the tumor were performed. Histopathology diagnosed a large aggressive angiomyxoma with uninvolved resection margins. Patient remains without recurrence at 8.0-months followup. Discussion and Conclusion: Non Aggressive angiomyxomais rare but should be excluded in any large vulval mass. We expect that awareness accompanied with wide free safe margin excision has important role to prevent the recurrence of tumor.