Abstract: Malformation of the craniovertebral junction area is congenital malformation of the occipital foramen and upper cervical vertebrae, which can present a variety of malformations of the bones, brain and spinal cord. There are still many controversies about the treatment of this disease. In recent years, with the interchange and development of multidisciplinary, there have been new progress and understanding of the treatment methods and concepts of the deformity of the craniovertebral junction area. Surgical treatment methods and the prevention of surgical complications of craniovertebral junction area were reviewed in this article.