Mesenchymal chondrosarcoma is a rare variety of chondrosarcoma. The localization in the head and neck is rare and mandibular involvement is exceptional. It is characterized by the formation of cartilage by the tumor cells. We report a rare case of a mesenchymal chondrosarcoma of the mandible, through this case, and in the light of the literature we will review the epidemiological, diagnostic and therapeutic aspects of this rare malignant tumor.