Background: angiomyofibroblastoma is a soft-tissue lesion. The vulvovaginal region of middle-aged females is the most frequent localization. Angiomyofibroblastoma is a well-circumscribed subcutaneous tumor at the vulva and perineum region. It is a painful and slow-growing tumor. It is often thought to represent a Bartholin's gland cyst. Treatment of choice is surgical excision.
Clinical case: We describe the case of a 49-year-old female with a right vulvar tumor that had been growing for 2 years. Ultrasonography, computed tomography and magnetic resonance revealed a tumor at the right ischiorectal fossa. Diagnosis after surgical excision was angiomyofibroblastoma.
Conclusions: angiomyofibroblastoma must be considered in the differential diagnosis of vulvovaginal tumors because its treatment differs from others lesions of that region.