BACKGROUND Smooth muscle tumors of the vulva are rare and sometimes create diagnostic difficulty because of unusual morphological features. METHODS A 29-year-old African-American female presented with a vulval mass during her 5th week of pregnancy. The lesion was surgically removed under the clinical impression of a Bartholin cyst. Histopathological findings of the lesion and immunohistochemical stain results are characteristic of myxoid epithelioid leiomyoma. CONCLUSIONS Up to now, there are only a few cases of myxoid epithelioid leiomyoma of the vulva reported in English literature. We review previously reported cases and discuss the morphologic differential diagnosis. The mass presented in this report was the largest benign epithelioid leiomyoma in the literature.