Aggressive angiomyxoma (AA) is a rare, slow-growing, and locally infiltrative mesenchymal tumor primarily affecting women of reproductive age, with a strong predilection for the vulvoperineal region. Despite its benign nature, AA has a high recurrence rate (30–72%), necessitating long-term follow-up. Diagnosis is often delayed due to its indolent growth and resemblance to other vulvar lesions. Surgical excision remains the mainstay of treatment, but complete resection is challenging due to its infiltrative nature, often requiring adjuvant hormonal therapy.

A 24-year-old unmarried nulliparous woman presented with a slowly enlarging, painless vulvar mass over three years. Clinical examination revealed a 5 × 4 cm non-tender, non-reducible lesion on the right mons pubis extending to the labia majora. The magnetic resonance imaging demonstrated a well-circumscribed, T2-hyperintense mass over vulva. The patient underwent wide local excision with negative margins, and histopathology confirmed AA with estrogen and progesterone receptor positivity. To minimize recurrence risk, three monthly doses of GnRH agonist (leuprolide 3.75 mg) were administered. No recurrence was observed over one year of follow-up.

AA is a rare tumor with high recurrence potential requiring surgical excision and prolonged follow-up. Adjuvant hormonal therapy, particularly GnRH agonists, may be beneficial in receptor-positive cases.