Primary epitheloid sarcomas of the vulva are rare but aggressive soft-tissu
e tumors. Most occur in Young women and are located in the region of Bartho
lin's gland. The histomorphologic and immunohistochemical features of the t
umor are characteristic. The differential diagnosis includes inflammatory c
onditions of Bartholin's gland. The tumors tend to recur locally. Wide loca
l excision is the treatment of choice.