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Extraskeletal myxoid chondrosarcomas are rare, soft tissue tumors with a characteristic histologic appearance. The majority of these tumors occur in the extremities. To our knowledge, this is the first reported case in the head and neck. As a group, extraskeletal myxoid chondrosarcomas behave in a less aggressive manner than do other forms of chondrosarcomas, but their clinical course can vary considerably. Surgery is the treatment of choice, and adjuvant radiotherapy or chemotherapy should be considered for cases that display a more cellular pattern and a less conspicuous myxoid matrix.