Clear cell sarcoma of the tendons and aponeuroses (CCSTA) is an aggressive, rare soft-tissue tumor representing approximately 1 per cent of all soft tissue sarcomas. Although it appears to be histogenetically related to melanoma, its clinical behavior resembles soft tissue sarcoma with a propensity for lymph node metastases. We report our experience at a tertiary referral center. Eight cases of CCSTA evaluated at the University of Illinois at Chicago's Medical Center between 1980 and 2002 were retrospectively reviewed. Patient data analyzed included age, gender, anatomic location, size of tumor, development of local, regional, and distant recurrence, and patient status at last follow-up. Local recurrence did not develop in any patient. Metastases developed in three patients with median time to presentation of metastases of 23 months (range, 8-36 months) from the time of initial diagnosis. Two of these three patients died of their disease with an average interval until death of 22 months from the time of initial diagnosis. The disease-free 2- and 5-year survivals were 68 per cent and 50 per cent, respectively. The overall 2- and 5-year survivals were 86 per cent and 68 per cent, respectively. CCSTA is an aggressive tumor of the soft tissues. These tumors present a high risk for development of distant disease and therefore warrant aggressive surgical management and treatment. Neither aggressive multiagent chemotherapy nor radiotherapy appeared to have an impact on outcome.