Mesenchymal chondrosarcoma of the head and neck is an uncommon tumor with a potential for exhibiting highly aggressive behavior. When these tumors arise in the head and neck region, they appear to have a predilection for the maxillofacial skeleton; less often, they may involve other soft tissue sites in the head and neck. The diagnosis is challenging and may be assisted by molecular pathologic techniques when only limited tissue is available for analysis. Management is primarily surgical. Although adjuvant radiation appears to convey some benefit by reducing tumor bulk when these lesions have extended beyond bony confines, there is no evidence to suggest that this is associated with improved outcome. Chemotherapy does not appear to be effective in the limited experience documented thus far. Patients with complete local control following resection should be followed closely for development of distant metastasis, which signifies a worse clinical outcome. Future effective therapy may be found in the identification of molecular targets responsive to adjuvant chemotherapy or biologic modifiers.