Aggressive angiomyxoma (AAM) is a rare mesenchymal tumor that usually occurs in the pelvis and perineum of young females. AAM can simulate Bartholin's gland cyst, abscess, lipoma, simple labial cyst, or other pelvic soft tissue tumors. Here we present five cases of AAM with mean age of 42. The patients mainly presented slow-growing mass in the abdomen and perineum (3 cases in the pelvis, 1 in the vulva, and 1 in the buttock). Color Doppler flow imaging revealed blood flow for the 3 pelvic lesions. Enhanced computed tomography and magnetic resonance imaging of the other 2 cases showed the typical “swirled” or “layered” structure characteristic. Through the pathological examination, its positivity to estrogen and progesterone receptors can justify enlargement and recurrence, confirming the tumor is AAM. All 5 patients underwent local tumor resection. Two patients recurred 8 and 15 months after surgery, respectively. The longest follow-up was 42 months. Although few cases are reported, early recognition demands high index of suspicion for both gynaecologists and pathologists. Wide surgical excision with tumor free margins is the basis of curative treatment. Adjuvant therapy may be necessary for residual or recurrent tumors. Long-term follow-up is recommended.