Craniocervical junction (CCJ) pathologies are diseases that often require incomprehensible definitions in neurosurgery practice, many classifications have been made in the literature, and treatment principles have not been clearly revealed. In this article, an effort has been made to approach the subject from a general point of view and to avoid complex details in order to better insight congenital CCJ pathologies by neurosurgeons. CCJ, whose embryological origin is determined by the first four occipital sclerotomes and first and second cervical sclerotomes, hosts many congenital pathologies in the developmental process. Occipital vertebrae, condylus tertius for occipital bone; atlas assimilation, hypoplasia, aplasia for atlas; for axis, os odontoideum, ossiculum terminale, odontoid aplasia are reviewed in general terms in this study. Considering the need for surgery regardless of pathology, CCJ`s congenital diseases are conditions that should be carefully evaluated in terms of instability and static compression, since compression of vital centers is in question. While posterior stabilization and fusion techniques are used in the presence of instability, anterior or posterior approaches can be used in case of compression on the neural tissue.