The article presents a case of a 12-year-old boy who was rushed to a hospital's neurology department due to untreated limping, progressive limbs weakness, repeated shoulder dislocation and progressive kyphosis to discuss morquio disease due to atlanto-axial subluxation. Various examinations revealed spastic quadriparesis, odontoid hypoplasia, and acetabular dysplasia. A cervical posterior decompression with occiput-to C2 fusion was proposed to treat his condition.