Background: Aggressive angiomyxoma (AA) is a rare vulvovaginal mesenchymal neoplasm with a marked tendency to local recurrence but which usually does not metastasize.
Case report: We describe a case of AA in the left labium majus pudendi in a 47-year-old woman who underwent incomplete surgical excision. Follow-up 2years later revealed no recurrence.
Conclusion: In the past, most authors advocated wide excision even if genitourinary and digestive tract resection were necessary. These days, a less radical surgery is recommended, but the significance of hormonal treatment and/or radiation therapy is not clear yet. Further investigation is necessary.