Aggressive angiomyxoma presenting as a vulval polyp

Aggressive angiomyxoma is a rare, locally invasive mesenchymal tumor, occurring predominantly in the pelvic-perineal region of adults and carries a high risk for local relapse and hence the need to differentiate it from the other mesenchymal tumors occurring in this region. Presentation as a pedunculated polyp, like in our case, is unusual for this rare tumor. Except for positive surgical margins, there are no clinical or histological means for predicting the tumor recurrence. A diligent long-term follow-up is mandatory. Though rare, this tumor needs to be considered in the differential diagnosis of vulval polyps.