A case of sclerosing epithelioid fibrosarcoma arising in the thigh of a 40-year-old man is reported. This recently described sarcoma has a distinctive histological appearance which may cause confusion with a variety of other sarcomas with an epithelioid pattern, and indeed with benign soft tissue tumours showing hyalinization. Despite its bland cytology, sclerosing epithelioid fibrosarcoma is capable of metastases, often several years after surgical removal. The patient described here developed lung metastases five years after excision and remains alive a further three years later. The differential diagnosis and possible histogenesis are discussed.