Leiomyoma of vulva is rare, and usually misdiagnosed clinically as Bartholin cyst. It usually presents spindle-shaped tumor cells, but some rare cases consisted mainly of atypical epithelioid tumor cells. We report here a case of 30-year-old woman consulting with a vulvar mass of 7 cm in the Bartholin glands area. The lesion was surgically excised with its capsule completely. Pathological examination and immunochemistry showed characteristic of epithelioid leiomyoma with myxoid stroma with both estrogen receptor (ER) and progesterone receptor (PR) staining negative, which was really rare as only 2 cases of vulvar leiomyoma with both ER and PR were reported before.