The craniovertebral junction (CVJ) is interposed between the unsegmented skull and the segmented spine; it is functionally unique as it allows the complex motion of the head. Because of its unique anatomy, numerous craniometric indices have been devised. Because of its complex embryology, different from that of the adjacent skull and spine, it is commonly the seat of malformations. Because of the mobility of the head, and its relative weight, the craniovertebral junction is vulnerable to trauma. Like the rest of the axial skeleton, it may be affected by many varieties of dysplasia. In addition, the bony craniovertebral junction contains the neural craniovertebral junction and its surrounding CSF: any bony instability or loss of the normal anatomic relationships may therefore compromise the neural axis. In addition, the obstruction of the meningeal spaces at this level can compromise the normal dynamics of the CSF and result in hydrocephalus and/or syringohydromyelia. To image the CVJ, plain X-rays are essentially useless. MR is optimal in depicting the soft tissues (including the neural axis) and the joints, as well as the bone itself. CT still may be important to better demonstrate the bony abnormalities.