Background: Aggressive angiomyxoma is a rare locally aggressive mesenchymal tumor of unknown etiology usually affecting the vulva, perianal region, buttocks or pelvis of reproductive age women.
Material: A series of three cases, one each of vaginal, vulval and labial angiomyxoma is being presented. The etiology, presentation, diagnosis and management of this rare genital tumor are outlined.
Conclusion: Angiomyxoma of vulva and vagina refers to a rare disease; diagnosis is not at all clinical, thus, cases presenting as bartholin cyst, benign vulval lesions and vaginal wall cysts should have complete radiological work up before excision, as pre-diagnosis can change the treatment modality and prognosis of patient.