Introduction. Due to its complex anatomy and function the craniovertebral junction (CVJ) is prone to several pathological conditions. The unique topography of neuro-vascular and bony structures makes surgical management of this area particularly challenging. Methods. A systematic online literature search was conducted in PubMed, Google Scholar, Scopus, and Web of Science with keywords relating to Chiari malformation, Wallenberg syndrome, Syringomyelia, and atlantoaxial dislocations. Results. This paper presents an up-to-date summary of the pathogenesis and surgical management of Chiari malformation, Wallenberg syndrome, syringomyelia, and atlantoaxial dislocation with particular emphasis on developmental and surgical anatomy. Conclusion. In such complex pathologies of the CVJ, the many factors contributing to the patient’s condition and the aspects playing a key role in the treatment process are all difficult to be considered concurrently. A better and universal surgical technique is impossible to be established.