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Dr. Lütfi Kırdar Kartal Eğitim ve Araştırma Hastanesi, Nöroşirürji Kliniği, İstanbul, Türkiye The craniovertebral junction has a complex anatomy and consists of the occipital bone and the atlas and axis vertebrae that surround the foramen magnum. Craniovertebral junction abnormalities may be related to the brainstem, cervical cord, cerebellum, cervical nerve roots, lower cranial nerves, and the vascular supply to these structures. In most cases, pathologic conditions affecting the craniovertebral junction become symptomatic after compromise of these neural structures. Signs and symptoms are varied but typically begin insidiously and arise fairly late, progress slowly, remain stationary, and rarely relapse. The complex development of this area requires an understanding of its embryological development and its relation to the patterning of the musculoskeletal system. Early anatomical descriptions of these pathologies will shed light on various syndromes that occur in this region. Anahtar Kelimeler : Craniovertebral junction, Abnormality, Embryology