The medical records and histologic material of 16 patients with extraskeletal myxoid chondrosarcoma were reviewed. The mean age of the patients was 52 years. Thirteen tumors arose in the lower extremity. Thirteen patients presented with primary, localized disease, whereas three presented with pulmonary metastases. Treatment of the primary site included wide excision or amputation in 13 patients and marginal or intralesional resections with radiation in three patients. The mean followup was 7.4 years. Five patients were continuously disease free (5- and 10-year event free survival 43% and 14%, respectively). Local recurrence developed in four, and metastases developed in six of 13 patients presenting with localized disease. Of six patients who received chemotherapy for systemic disease, four had disease progression and died, and two had a response to chemotherapy (one partial, one complete). The mean survival after onset of metastases was 45 months. Overall 5- and 10-year survival was 87% and 63%, respectively. The current series suggests that extraskeletal myxoid chondrosarcoma is an intermediate-grade neoplasm with a tendency toward recurrence and metastasis. Survival after relapse may be prolonged. More effective therapy for systemic disease is needed.