Chordomas are rare tumours that arise from remnants of the embryonic notochord. They can be located in all places where the notocord existed, such as the nasopharynx, clivus, sella turcica, foramen magnum, cervical spine and sacrococcygeal region. This chapter will focus on chordomas affecting the craniovertebral junction (CV). Chordomas are rare malignant tumours with an incidence of less than 1 per 100,000. The site of origin of skull base chordomas is usually the clivus and from here they can extend into the anterior fossa, middle fossa, the sellar and parasellar regions, and through the foramen magnum into the upper cervical spine. In the majority of cases the typical histological appearances and characteristic location of chordomas render the diagnosis straightforward. The symptoms and signs are highly variable and depend on the exact site of the lesion and the involvement of adjacent structures. These can range from cranial nerve palsies, motor and sensory deficits, to gait disturbance and balance problems. Radiological diagnosis of craniovertebral junction chordomas has improved with the advent of modern CT and MR imaging modalities and tumour location and extent of skull base involvement can be precisely defined. Surgery is the most effective form of treatment for patients with craniovertebral junction chordomas and radical resection augmented with adjuvant therapies such as proton beam therapy have been shown to prolong survival. Although chordomas are usually slow-growing tumours they are locally aggressive with a tendency to infiltrate adjacent tissues and organs. Local recurrence is common and often causes death. Metastasis is well documented but is uncommon. This chapter will aim to give an overview of the diagnosis and management of CV junction chordomas.