A rare case of epithelioid sarcoma of the coccyx is described. A 23-year-old man had coccidynia for 2 years, becoming permanent. The patient underwent an "en bloc" excision including a resection of the extremity of the coccyx. Postoperatively, six cycles of a chemotherapy combining ifosfamide and adriamycine were alternated with a 60 Gy external beam radiotherapy. The patient remains free of disease 9 months after the operation. We review the clinical characteristics and treatment of epithelioid sarcoma.