A malignant xanthogranuloma located in the mandible presented as a tumefaction of the alveolar crest with labiomental hypoaesthesia in a 52-years-old woman. The patient was treated with combined chemoradiotherapy and remained in complete remission for 14 years. Epidemiologic features (age, sex, race) of this tumour are discussed in light of the 24 cases reported in the literature. Histology features and possible treatment protocols as well as observed results are discussed. Five-year survival rate in malignant cervicofacial xanthogranulomas is estimated at 48%.