A 54-year-old woman was found to have a right adnexal mass during routine pelvic examination. A total abdominal hysterectomy and salpingo-oophorectomy were performed. A well circumscribed, 2.5 cm right ovarian was composed of small spindle and epithelioid cells in solid perivascular nests. The tumor cells were reactive for vimentin and smooth muscle actin. Ultrastructurally, pinocytotic vesicles and basement membrane were prominent. This is the second reported case of glomus tumor of the ovary and the third occurring in the gonads.