The clinical significance of abnormalities of the craniocervical junction has gained tremendous momentum with the increasing interest in the area as a result of better neurodiagnostic imaging. Abnormalities of the bone, soft tissue, and neural structures are easily recognized and there is a better understanding of the biomechanics of this complex region as well as the embryology. A database of symptomatic patients, children, and adults with neurological symptoms and signs secondary to abnormalities at the craniocervical junction have been evaluated by the author at the University of Iowa Hospitals and Clinics. This large database comprises 6,000 patients and provides a better awareness of the natural history of abnormalities as well as diseases affecting this area.A surgical physiological approach to pathology at this region was proposed in 1977 and still holds true. However, with the advent of better imaging as well as surgical approaches and instrumentation, treatment of problems in this region can be divided into the clinical syndromes, surgical approaches, and techniques of stabilization. A brief outline of the evolving nature of this is provided in this manuscript.