A case of epithelioid sarcoma localized in the palm of the left hand in a 32-year-old woman is reported. The tumor evolved for many years, clinically and microscopically simulating palmar fibromatosis. Epithelioid sarcoma is an uncommon malignant tumor, often misdiagnosed by surgeons and pathologists. It occurs in young patients and is often localized at the upper distal extremity. Microscopically, epithelioid sarcoma shows nodules manifesting fibrous hyaline cores with central necrosis. It contains epithelioid and spindle cells immunoreactive to keratin, epithelial membrane antigen and vimentin. Recurrences, lymph node metastases and lung metastases are frequent. Surgical literature tends to recommend wide "en bloc" excision or amputation, combined or not with adjuvant radiation therapy.