In this report, we share our experience of a case of maxillo-facial extraskeletal myxoid chondrosarcoma, a very rare location for this neoplasm. In addition, a literature review is provided. The patient, a 61-year-old male, had a maxillary mass encroaching on the nasal cavity. After debulking, the tumor recurred, attaining its pre-surgical proportion in two months. The patient improved clinically with radiation and remained stable for about one year. However, he ultimately developed metastases in his lung which were treated with palliative chemotherapy with a good outcome lasting three months. We could find only eight reported cases of this tumor in the head region of which two are in the maxilla; hence, ruling out other primary sites is mandatory for a patient presenting with a primary head and neck mass. Surgical removal may be complicated because of the location. A combination of surgery and radiation is the management of choice, with palliative chemotherapy in metastasis.