Epithelioid sarcoma is a relatively rare soft tissue tumor of uncertain histogenesis that mainly occurs in the hands and forearms. It frequently has a benign appearance and slowly growing nodules that tend to ulcerate and recur after excision. Distant metastasis usually occurs quite late in the course. We observed an epithelioid sarcoma in the anterior mandibular gingiva that metastasized to cerebellum of a 56-year-old man. A review of the literature revealed no other reported cases of epithelioid sarcomas in this region. This report records a unique primary site for this tumor. The diagnosis was difficult because this tumor is often confused with a variety of benign and malignant conditions, owing to its clinical and histopathological presentations.
