A case of chondrosarcoma involving the maxilla is reported. The salient points of the presented case include; diagnostic and pathologic processes, radical surgery and simultaneous chondral graft and finally long terme follow-up.The most important lessen to be learned from this report, first is that the patient referred too late in the course of the disease, thus, the tumor was progressively involved entire maxilla ultimately. In the literature, in more knowledged countries usually the size of tumor is reported to be between 2-4 cm. Secondly, Histophthology study plays an important role in the survival rate and final patient’s outcome. The microscopic differential diagnosis of chondrosarcom from osteoma, chondroma, osteoid osteoma, fibro-osseous lesions and osteogenous sarcoma is an important and often difficult consideration. The most oral and maxillofacial pathologists believed that the specimens is better to be large and multiple, so provide a more suitable overview by microscopic study.Finally wide resection of the chondrosarcoma is the most acceptable choice of treatment. The role of radiotherapy and chemotherapy as an adjunctive treatment remain controversial. On the basis of the extremely aggressive clinical behavior of these tumors, the survival rate is rather poor.Key words: Chondrosarcoma, Maxillofacial Surgery, Treatment and Follow up