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Aggressive Angiomyxoma (AA) is an uncommon, locally infiltrative mesenchymal tumor that primarily originates from perineal and pelvic sites of women, particularly in the 4th decade of life with having an emphasized tendency for local recurrence, whereas it has a low tendency to metastasize. Patients often present with nonspecific symptoms such as painless visible mass that might be misdiagnosed with every mass in genital and pelvic sites in reproductive-age women.

We describe a 31-year-old female presenting with an enlargement on the right labia majora. Ultrasound and magnetic resonance were carried out, and the mass was surgically excised completely and without complication. The diagnosis of AA was made based on characteristic histopathological features. The postoperative follow-up for recurrence is currently being continued.

Due to …