Testicular metastasis from sarcomas is extremely uncommon. Metastasis from extraskeletal myxoid chondrosarcoma to the testis has not been previously reported. We report a case of extraskeletal myxoid chondrosarcoma that, 4 years after surgery, manifested with testicular enlargement, a period punctuated by three local recurrences. The testicular tumor showed pathologic features similar to the previous specimens, and metastatic myxoid chondrosarcoma was diagnosed. This case demonstrates that even low-grade sarcomas can metastasize to rare sites, such as the testis, and pose a diagnostic dilemma. To the best of our knowledge, this is the first case of extraskeletal myxoid chondrosarcoma with metastasis to the testis.