Antlanto-axial dislocation with blocked and ankylosed C2-4 vertebrae causing severe canal stenosis with compression of cervico-medullary junction has been reported. The knowledge of such anomaly may be importance for radiologists, anaesthesiologists, orthopaedicians and neurosurgeons because cranio-cervical junction anomaly may result in sudden unexpected death. It can also result in dysphagia, dysarthria due to compression of cranial nerves and quadriparesis due to compression of spinal cord. We here report a rare case of cranio-vertebral junction anomaly with blocked and ankylosed C2,3,4vertebrae with evidence of atlanto-axial dislocation causing compression of cervico-medullary junction and leading to cervical myelopathy.International Journal of Human and Health Sciences Vol. 03 No. 01 January’19. Page : 40-42