Primary chondrosarcomas of the head and neck region are exceedingly ra
re neoplasms. These lesions have clinical and histologic characteristi
cs that make them difficult to discern from benign chondromas. Additio
nally, they may arise from the periosteum, making it even more difficu
lt to distinguish them from osteosarcomas. We present a patient with a
periosteal chondrosarcoma of the maxilla. Characteristic radiologic a
nd histologic findings associated with this lesion are described This
is the first reported case of such a lesion in the maxilla, as previou
s reports of periosteal chondrosarcomas have involved long bones. The
diagnostic dilemma posed by this extremely unusual lesion is discussed
as well as treatment options.