Aggressive angiomyxoma is a rare mesenchymal tumor that most commonly arises in the vulvovaginal region, perineum, and pelvis of women. The term aggressive emphasizes the often infiltrative nature of the tumor and its frequent association with local recurrence. Patients often present with nonspecific symptoms which are frequently misdiagnosed with more common entities, such as a Bartholin cyst, lipoma, or hernia. Histologic examination reveals a hypocellular and highly vascular tumor with a myxoid stroma containing cytologically bland stellate or spindled cells. The tumor cells are characteristically positive for estrogen and progesterone receptors, suggesting a hormonal role in the development of the tumor. Chromosomal translocation of the 12q13-15 band involving the HMGA2 gene has been described. Surgical excision is the treatment of choice, although treatment with gonadotropin-releasing hormone agonists is an emerging therapy. Metastases are exceedingly rare, and overall, the prognosis is good.