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<p>Aggressive angiomyxoma is a rare soft tissue neoplasm that usually arises within the perineum. It often occurs as a vulvar mass and clinically simulates a Bartholin's gland cyst. Most patients are in the second or third decade of life, but some cases have also been reported in children. This is the report of a 21 year old woman with 4.5 × 3 × 1.5 cm mass in right labia major. The patient underwent wide local excision surgical treatment. Histological examination showed high vascular myxoid tumor containing spindle cells. Immunohistochemical study of cells showed positive reaction to estrogen and progesterone and negative reaction to S100, SMA and desmin. Treatment with a gonadotropin-releasing hormone agonist was administered to deal with residual tumor and prevent local recurrence for 6 months.<br /><strong>KEY WORDS</strong>: Aggressive angiomyxoma, vulva, pregnancy, Gonadotropin releasing hormone agonist.</p>