A broad definition of instability of White and Panjabi incorporates nearly all pathological conditions in the upper cervical spine and craniovertebral junction (CVJ) including trauma, sterile inflammatory processes, infections, congenital deformities, tumors, as well as iatrogenic surgical interventions-all potentially resulting in instability. Based on experience with more than 200 surgically treated patients with CVJ and upper cervical spine instability, we are presenting an overview of currently available and acceptable methods of stabilization of this region. Various techniques of upper cervical spine stabilization have been described. The history of C1-2 surgical stabilization started in 1891 when Hadra used a wire loop to stabilize the cervical spine posteriorly for Pott's disease. A well-known technique of Gallie (1932) using bone grafts fixed to the posterior arches of C1 and C2 was further developed by Brooks, Jenkins, and Sonntag in order to adapt the shape of the graft to the arch interspace and increase the stability of the fixation. In 1979 Magerl first used the transarticular C2-1 fixation. Because of the risk of vertebral artery injury, alternative techniques were developed by Goel (1988) and Harms (2001). These last-described methods, supplemented with Gallie-type of grafting, represent the most frequently used techniques today. They offer immediate three-dimensional stability and thus no external support is necessary. The long-term fusion rates are more than 90% in the majority of published series. Reports of occipitocervical (OC) arthrodesis are less frequent. In 1927, Forester described noninstrumented OC fusion with fibular strut graft for atlantoaxial instability caused by odontoid fracture. Techniques of OC stabilization were developed concurrently with those above and the most difficult issue was the fixation of the devices and grafts to the occiput. Wires, screws, rods, and even bone cement were used to attempt stable OC constructs. Currently, modern modular titanium alloy systems are available, allowing us to simply fuse occiput to any part of the cervical spine. Finally, the isolated transarticular C1/0 screw fixation was described by Grob (2004), allowing monosegmental stabilization in rare C0/1 instability.