Injuries to the upper cervical spine (C0-C2) play a major role in surgical treatment of traumatic sequelae in the entire cervical spine. Even though the number of such operations has increased in recent years, there are no clear treatment recommendations for most types of cervical spine injuries. In view of the wide range of injury types and the correspondingly large number of treatment options, this review focuses mainly on the following types of injuries: C0 fractures, occipital condyle fractures (OCF), atlanto-occipital dislocation (AOD), atlas fractures, atlantoaxial dislocation (AAD), and axis fractures. Important aspects of the mechanisms of injury, clinical signs and symptoms, diagnostic procedures, and treatment options are discussed. Special emphasis is placed on comparatively reviewing the different treatment options discussed in the literature. A summary in table form is presented at the end of each chapter for quick reference.