Mesenchymal chondrosarcoma (MCS) is a rare malignancy that was first described by Lichtenstein and Bernstein in 1959. This paper reports the latest case of mandibular MCS and provides a review of cases of mandibular MCS from 1971 to the present. The literature was searched using the PubMed and Ovid engines; bibliographies were cross referenced. A metaanalysis of the reported cases was performed and Kaplan-Meier curves generated for all treatment options. In the paediatric population, MCS is a rare and aggressive tumour. Since 1971, only 15 cases of MCS have been reported in the mandible of patients less than 20 years old. Surgical treatment positively impacts survival and is not improved with radiation or chemotherapy. Local recurrence was frequent, aggressive and dramatically affects survival. This meta-analysis shows that primary intervention with surgery remains the standard of care for MCS. The role for chemotherapy and radiation therapy in the treatment ofMCSremains unclear. © 2009 The Authors Journal compilation © 2009 Blackwell Munksgaard.