A case of malignant rhabdoid tumor of the vulva in a 25-year-old female was examined. The patient presented with a subcutaneous nodule in the left labium majus. Smears of the material obtained by percutaneus fine-needle aspiration demonstrated clusters of atypical cells with prominent nucleoli. The tumor measured 6 × 5 × 5 cm and appeared tan to brown on the cut surface and partly cystic. Pathological findings obtained from intraoperative frozen tissue sections had been originally interpreted as rhabdomyosarcoma. Light microscopic examination revealed that polygonal tumor cells having vesicular nuclei with prominent nucleoli were arranged in sheets and the great majority of the tumor cells contained an eosinophilic globular paranuclear cytoplasmic inclusion. Ultrastructurally, this cytoplasmic inclusion corresponds to whirls of intermediate filaments. Vimentin immunoreactivity was detected in both the cytoplasm and cytoplamic inclusion of almost all the tumor cells. No cytokeratin and desmin immunoreactivity were detected in the tumor cells. The Ki-67 labeling index was 36% and the DNA content of the tumor cells, which was examined by image cytometry, demonstrated diploidy (DNA index = 0.95).