The occipito-cervical junction (OCJ) is defined as the transitional zone between the occipital cranial base and the upper cervical spine. It includes the occiput (C0), the atlas (C1) and the axis (C2). This region is submitted to high biomechanical stresses, being the junction between the “heavy” head and a long lever arm, the spinal column. That is the reason why this junction is frequently involved in trauma, being the fractures the most frequent pathology at that level. During my residency program, I developed a strong interest on the complexity of OCJ and on the pathologies involving it. Benign tumors, metabolic diseases and traumatic fractures of the OCJ have been the topic of several publications from within the Departments of Neurosurgery of Naples and Geneva. The following thesis is a summary of my personal interest on the OCJ.