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Highlights • The temporo-mandibular joint chondrosarcoma is extremely rare, and can be misdiagnosed as parotid gland tumor. • Surgery is standard treatment with the aim of preserving the temporo-mandibular joint function. • Adjuvant radiation is used in certain cases to improve local control., Introduction Chondrosarcoma is a malignant tumor originated from cartilage cells. The most common sites of chondrosarcoma are pelvis, femur and ribs. The temporo-mandibular joint (TMJ) chondrosarcoma is extremely rare, with approximately 30 cases reported in worldwide. Presentation of case In this report, we present a case of chondrosarcoma in the left TMJ, which was successfully treated with surgery and radiation therapy. A 47 year-old women was admitted to our hospital with a mass in left pre-auricular region. On examination and para-clinical test results, the initial diagnosis was parotid gland cancer. In operation, the lesion was a mass originating from the left TMJ. Tumor resection was performed with close margin. The post-operative pathological result was well-differentiated chondrosarcoma. She was indicated adjuvant radiation therapy due to inadequate surgical therapy. There was no evidence of recurrence after 6 months follow-up. Conclusion The TMJ chondrosarcoma is needed to distinguish from other diseases especially parotid gland tumor. Surgery and adjuvant radiation therapy are standard care with the aim of preserving joint function.