- Aggressive angiomyxoma (AA) is an uncommon, slow growing, locally infiltrative but non-metastasizing, distinctive mesenchymal tumor that predominantly affects the pelvis and perineum of premenopausal women. The mainstay of treatment is local excision with tumor-free margins; however, recurrences are common and related to inadequate primary excision. A pedunculated 3-cm mass arising from the vaginal suburethral area in a 49-year-old premenopausal woman was resected around the base of its pedicle. Microscopic examination revealed numerous blood vessels of various sizes set in myxoid stroma with spindle shaped fibroblasts. Immunohistochemical staining was strongly diffusely positive for vimentin, desmin, estrogen receptor (ER) and progesterone receptor (PR), weakly focally positive for CD34, and negative for S-100 protein, actin and Ki-67. These findings are compatible with the diagnosis of AA. To date, six months after surgery, the patient is alive and without evidence of recurrence. AA is often clinically misdiagnosed and it is only the microscopic examination strengthened with immunohistochemical staining that definitely and undeniably contributes to the final diagnosis of AA. Based on this case report and on the previously reported five cases of pedunculated AA arising from the vulvovaginal region, including one tumor arising from the vaginal suburethral area, it seems that pedunculated AAs arising from the vulvovaginal region are at negligible risk of recurrence after local excision.