Chordomas and chondrosarcomas are located in any part of the spine, skull base and clivus. They are malignant lesions. They may progress with varying degrees of local recurrence depending on the degree of differentiation. Therefore, subtotal resection may be preferred to avoid serious damage to neural structures and regional muscle and bone tissues during excision. The main treatment protocol for both pathologies is total resection as much as possible and radiotherapy. Chemotherapy, molecular targeted therapies, and proton beam therapy are reported as potentially beneficial.