MATERIAL and METHODS: The Craniovertebral junction (CVJ) is structurally a composite of many bone and ligamentous structures, yet functionally, it is a stable interlocking unit that acts as a transition between the skull and spine. Due to the mobile junction of upper cervical spine and the complex transition between the spinal cord and brain, CVJ has a complex developmental anatomy. Thus the bony abnormalities of the CVJ involve not only the osseous structures but also the encompassed nervous and vascular system. When the abnormalities in this region are symptomatic, surgeon need to be well familiar to the functional anatomy, biomechanics and embryology of the region for an efficient treatment.

RESULTS: Management of these anomalies depends on the better recognition of the underlying pathologies that affect this region by means of the appropriate radiologic diagnostic tools. The surgical management of CVJ instability in pediatric patients presents unique challenges.

CONCLUSION: Diminutive osseous and ligamentous structures, as well as the peculiar configuration of vertebral components, the presence of congenital malformations with possible abnormal vertebral artery location, and the need for external immobilization and the potential for its complications have made it important to critically evaluate instrumentation and its incorporation into the management of pediatric patients who require craniocervical stabilization. The surgeon is referred to management protocol regarding the treatment algorithm for problems in CVJ. Anahtar Kelimeler : Craniovertebral junctions anomalies, Atlanto-occipital dislocation