Purpose: High grade variant of mesenchymal chondrosarcoma in mandible is a very rare tumor documented in years. Hence, we have performed analysis of the reported cases of high grade variant of mesenchymal chondrosarcoma with emphasis on its treatment modalities. Material and Methods: We report a case of a 25 year-old man with pain and swelling which was in rapid onset in nature on right body region of mandible. This review also includes the two similar type of cases and analyzed the data of the patients, lesion site, treatment approach and events of recurrence. Results: Only three patients of high grade variant of Chondrosarcoma of jaws has been reported till date including this case. The primary treatment in all patients was radical surgery consisting of resection with clear margins. Out of this 2 patients were undergone for radiotherapy. Conclusions: This article highlighted the potentially aggressive biological behavior of high grade variant of mesenchymal Chondrosarcoma. Inspite of surgical resection with clear margins and giving radiotherapy possible outcome was not achieved. However, due to the rarity, more well-documented cases with long follow-up periods are needed in order to further define the optimal treatment modalities and prognosis.