Most cases of primary malignant tumors of bone occur in children and adolescents. There are several types to be classified according to clinical and radiological findings and histologic diagnosis Ewing's sarcoma and osteogenic sarcoma; chondrosarcoma, malignant tumor of cartilage, is also classified in this group of tumors. Incidence, age-and-sex-distribution clinical presentation, and diagnostic procedures are reported for the different types of tumors, with special reference to primary localization in the skull. Different therapeutic modalities are discussed. Cure rates in these highly malignant tumors are still low, but evidently improving.