The craniovertebral or craniocervical junction (CVJ) is a transition zone between mobile cranium and relatively rigid spinal column and refers to the occiput, atlas, axis and supporting ligaments. It encloses the lower cerebellum, medulla, spinal cord and the lower cranial nerves. Anomalies of CVJ include a number of pathologies, mostly developmental. Anomalies alone may not always cause symptoms. Associated basilar invagination and A-A subluxation are the most common symptomatic lesions involving CVJ. Adequate reduction, if necessary with anterior decompression and stabilization are widely employed with good outcome. They are discussed in this chapter along with a brief description of acquired pathologies.