We report the case of perineal aggressive angiomyxoma in a 46-year-old woman presenting as left perineal swelling associated with superficial dyspareunia. Initial clinical examination revealed a 4- to 5-cm cystic mass in the posterior aspect of the left labia majora, not thought to be typical of a Bartholin cyst. A magnetic resonance imaging (MRI) scan revealed a well-defined 2- x 1.5- x 2-cm area posterolateral to the lower vagina on the left but anterolateral to the anal canal extending into the left ischiorectal fossa, with no obvious involvement of the anal sphincter complex. Excision biopsy was performed via an incision in the left labia majus under general anesthesia. Histologic findings were consistent with aggressive angiomyxoma of the vulva. This was confirmed by immunohistochemistry showing spindle cells positive for vimentin with strong nuclear staining for estrogen and progestogen receptors. Postoperative management following discussion at the multidisciplinary gynecological oncology meeting was to perform a repeat MRI scan 6 weeks postoperatively, and treatment with raloxifene was commenced for its antiestrogenic property.