Chondrosarcoma is a malignant cartilage-forming tumour of bone, of which distinct clinicopathological subtypes are known. Conventional chondrosarcoma is notorious for its locally aggressive behaviour as well as for its resistance to chemotherapy and radiotherapy; so far surgery is the only effective therapeutic option. During the past 10 years, substantial new insights have been gained about molecular cell biology, molecular cytogenetics, and immunopathology, leading to better understanding of chondrosarcoma development at the molecular level, which will ultimately lead to better clinical understanding and possibly to the development of targeted treatment.