RESUMO
Lipomatous variant of angiomyofibroblastoma is a rare tumor of the vulva. Most of cases are usually premenopausal and present with a slowly growing mass without pain. A 46-year-old woman presented with a swelling of the left labium major. The lesion was thought to be a Batholin's gland cyst or a lipoma preoperatively and a local excision was performed. The tumor was well demarcated and rubbery. On microscopic examination, the alternating hypercellular and edematous hypocellular component with abundant vessels was noted. A large amount of adipose tissue was scattered in the lesion. Stromal cells were positive for vimentin, estrogen receptor, and progesterone receptor and negative for desmin antibody immunostaining. The local excision was enough for complete treatment. No recurrence was noted for 7 months after surgery.