Aggressive angiomyxoma of vulva is a benign, slow-growing tumor originating from myxoid connective tissue cells. Multiple local recurrences of the tumor are known to occur, and its propensity to spread is minimal. There currently exist just about 350 cases that have been reported in the scholarly literature.

We report a case of a 40-year-old Indian married woman from Maharashtra, who presented with a swelling on the right labia minora for 2½ months. She gives history of a similar swelling over the same site, diagnosed as a Bartholin cyst and marsupilized. Local examination showed a polypoidal fleshy mass originating from right labia minora with multiple nodular cystic swellings present in the mass. After taking informed written consent, a well-defined mass lesion of 5 × 3 cm size with smooth, lobulated margins, arising from right upper 1/3 of labia minora was excised from base under general anesthesia. Histopathology was suggestive of aggressive angiomyxoma. Immunohistochemistry was done, which was positive for estrogen and progesterone receptors. Her postoperative recovery was uneventful.

One of the differential diagnoses for vulvovaginal growth in a female is aggressive angiomyxoma. Considering the tumor is known to cause local recurrences, treating patients with it requires a multidisciplinary approach, accurate diagnosis, and proper care.