Aggressive angiomyxoma is a rare, locally infiltrative soft tissue tumor that usually arises in the vulvoperitoneal region of young female. Frequent relapses are common. Clinicians should consider the diagnosis of aggressive angiomyxoma when a patient presents with an atypical vulvoperineal mass, because an incorrect diagnosis may lead to repeated surgical procedures. Treatment is wide surgical excision. Medical management with a GnRH agonist and radiation therapy with total dose of 60 Gy may be helpful adjuvant treatment in recurrent aggressive angiomyxoma. We experienced a case of recurrent aggressive angiomyxoma and report it with a brief review of literatures.