Background: Aggressive angiomyxoma is an uncommon mesenchymal tumor that preferentially involves the pelvic and perineal regions of females. Since its initial description in 1983, approximately 65 cases have been reported in the English literature.

Case: A 33-year-old parous woman with a slow growing pedunculated soft tissue tumour of the vulva which later underwent surface ulceration. Pathological findings in the case were suggestive of an aggressive angiomyxoma, a distinctive soft tissue non-malignant tumour, which could, at times be locally aggressive.