Management of congenital and acquired diseases of the craniovertebral junction (CVJ), comprising the occipital bone surrounding the foramen magnum, the atlas, and the axis vertebrae, constitutes one of the more complex areas of neurosurgery. This is particularly true in the pediatric population, in which the neurosurgeon is faced with a wide variety of congenital developmental anomalies and acquired diseases and must take into account the need to avoid significant interference in still growing structures. Pediatric neurosurgeons are increasingly called upon to face this stimulating region, the diseases of which may now be managed with rewarding results even in the first years of life owing to progress in the neurosciences, availability of advanced neuroimaging diagnostic tools, and improved surgical and anesthesiological techniques. This dedicated volume in the series Advances and Technical Standards in Neurosurgery (ATSN) provides a comprehensive approach to CVJ diseases and their management based on the multidisciplinary cooperation of neurosurgeons, anatomists, neuroradiologists, and neuroanesthesiologists. The contributing authors represent the most renowned clinical and surgical experts from Europe and beyond. In accordance with the traditional structure of ATSN, the 12 chapters of the volume are organized into advanced (7) and standard (5) contributions. The main topics highlighted are embryology, normal and abnormal development of the CVJ, including the related vessels, modern radiological contributions to diagnosis, genetic and metabolic factors which may impact on the surgical strategies, the opportunities offered by traditional operative techniques, and the recently introduced minimally invasive and endoscopic surgical modalities. Special emphasis is also placed on the evolution of the principles of surgical treatment as matured during the past decade by experiences in the still open field of pediatric neurosurgery.