Clinical manifestations associated with atlantooccipital fusion are mainly attributed to the ligamentous laxity of the transverse ligament in relation to the odontoid process. The flexion and extension movements of neck lead to the compression of spinal cord. The chances of regeneration of the central nervous system decreases with increasing age and repeated injuries to the nerves from the odontoid may result in the neurological symptoms. Atlantooccipital fusion is associated with narrowing of foramen magnum, compression of spinal cord or brain stem [12]. Serious clinical manifestation such as myopathy, limitations of neck movements, muscular weakness and atrophy and sensory loss may be associated.