Chondrosarcoma is an uncommon malignant mesenchymal tumor characterized by the production of cartilaginous tissue and the absence of production of bone tissue. Maxillary and mandibular localizations are extremely rare and have a poor prognosis. The clinical and radiographic findings are similar to those seen in other tumors of the jaw, often delaying diagnosis and treatment and subsequently jeopardizing prognosis. In the literature, the mean delay from first clinical signs to diagnosis is about 8 months. As in all tumoral diseases, pathology confirms the diagnosis. We report a case of chondrosarcoma of the maxillary bone and review the literature, focusing on the difficulty in establishing the diagnosis, even at the pathology examination. We propose wide surgical excision, which, in agreement with other reports in the literature, is the only therapeutic possibility. We observed no sign of recurrence at 5-year follow-up. Radiotherapy and chemotherapy may be useful in certain cases.