Background: Aggressive angiomyxoma (AA) is a rare and slow-growing tumor in the pelvic and perineal regions that might develop into other perineal structures. It can present variably, ranging from a painless mass to non-specific symptoms such as dyspareunia. Due to the high relapse rate, extensive tumoral resection is reasonably required to prevent recurrences. It is also commonly confused with other conditions such as lipomas, Bartholin’s gland cysts, and hernias.

Case Presentation: A 43-yr-old female diagnosed with AA 10 yr ago was evaluated as a consequence of the tumor recurrence. She presented rare manifestations of a giant and cystic pelvic mass involving pararectal and paravaginal tissue in front of the sacrum.

Conclusion: Although AA is a rare and slow-growing tumor, close observation is recommended due to the high relapse rate. Furthermore, extensive tumoral resection and regular follow-up can reduce morbidity in these patients.

Key words: Vulvar neoplasm, Perineum, Pelvic neoplasms, Recurrence.

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