The management of our patient was similar to that of many patients with soft tissue sarcomas. His primary care was in a General Hospital where the surgeon had no specialist experience of these tumours and did not anticipate such a diagnosis. As a result the diagnostic procedure was inappropriate and compromised the chances of success of further more radical conservative surgery. Radiotherapy was unable to prevent local recurrence and amputation became necessary. Pulmonary metastases developed and were resected but within a short space of time extensive inoperable metastases recurred. Although these responded to chemotherapy, the response duration was short and the patient died soon afterwards. The major lessons to be learned are the need to have a high index of suspicion about a possible neoplastic cause of increasing soft tissue swellings and to refer patients for biopsy to a specialist centre where there is experience of all aspects of the management of these rare but potentially curable malignancies.