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Bahçeşehir Üniversitesi, Beyin ve Sinir Cerrahisi Anabilim Dalı, İstanbul, Türkiye Malformations of craniovertebral junction are quite complex patologies. They occur as a result of defects during embryogenesis. In this chapter we aimed to explain the embryology of craniovertebral junction for a better understanding of this region malformations. Anahtar Kelimeler : Craniovertebral junction, Congenital malformations, Embryology