A case of epithelioid sarcoma in a young man is described. At 16 years of age the patient had two skin ulcers removed from his hand, which were diagnosed as synovial sarcoma. At 21 years he underwent wide excision of the same area, including part of the thenar muscle, and a diagnosis was made of palmar fibromatosis with no confirmation of malignancy. Histological examination of biopsied growths three years later at age 24 showed the typical picture of epithelioid sarcoma. Following further excisions of local recurrences and removal of half the distal part of the forearm, the patient agreed to below-elbow amputation at age 27. He died within the year. The case presents the typical features of epithelioid sarcoma: apparently benign presentation on the hand in a yound man, a histological picture resembling other diseases resulting in misdiagnosis early in the disease, and local recurrences and metastasis. The misleading clinical and histological pictures of this insidious disease are discussed.