The poor prognosis of malignant rhabdoid tumor is emphasized and histopatho
logical criteria for distinction from epithelial sarcoma of the vulva are d
iscussed. Immunohistochemical analyses were performed by using nine differe
nt antigens including vimentin, cytokeratin, epithelial membrane antigen, c
arcinoembryonic antigen, desmin, muscle-specific actin, S-100 protein, AP-1
5, neuron specific enolase. This is the sixth reported case of a malignant
rhabdoid tumor of the vulva. The patient died eight months after the initia
l diagnosis in spite of a combination of surgery, adjuvant chemotherapy and
external radiotherapy.