Epithelioid sarcoma is a rare distinctive malignant neoplasm of soft tissue with frequent recurrences and late metastases. It typically affects young adult males, and epithelioid sarcoma arising in the first decade of life is very rare. Epithelioid sarcoma may be confused with a wide range of benign, especially granulomatous, diseases and other malignancies, but the differentiation can be made, in general, by the characteristic clinical, histopathologic, and immunohistochemical features.