Achondroplasia represents the common variety of skeletal dysplasia causing short-limb dwarfism. It may be associated with congenial craniovertebral junction anomaly, including atlantoaxial dislocation, Os odointoideum or basilar invagination or acquired as a result of the previous foramen magnum region surgery. However, long segment cervical compression from the first cervical vertebra region to sixth cervical region, such extensive compression producing myelopathy is not reported, and current case represents first case in the western literature. He was managed surgically with a good outcome. Perioperative management along with brief review of pertinent literature along with the management of such case is discussed in brief. Anesthetic management of such patient with coexisting extensive cervical compression presents unique challenges in view of the multisystem involvement along with preexisting anatomical and physiological constraints.