Abstract. Cementoblastoma is a form of benign odontogenic tumor, with the preferred treatment consisting of tooth extraction and follow-up examinations, while in certain cases, follow-up examinations without surgery are performed. Osteosarcoma of the jaw is a rare, malignant, mesenchymal tumor, associated with a high mortality rate and low incidence of metastasis. Cementoblastoma and osteosarcoma of the jaw are dissimilar in terms of their histological type and prognosis; however, there are a number of covert associations between them. The present study describes the case of a 20-year-old female with an unusual swelling in the left mandible that had been present for 2 years. The patient was initially clinically diagnosed with cementoblastoma; however, following pathological examination, a diagnosis of osteosarcoma was confirmed. A total mandibulectomy was performed on the left mandible, with a fibular osteomyocutaneous flap used for reconstruction. At the 6-month post-operative examination, the patient demonstrated normal occlusion and oral commissure, and no recurrence or metastasis was observed over 2 years of follow-up.