The aim of this paper is to show that osteochondromas of the cervical vertebrae can cause myelopathy and neck pain. The reported etiology, diagnosis, treatment and differential diagnosis were reviewed. Osteochondromas may present as a solitary lesion with no genetic component or as multi ple lesions as a part of a genetic disorder known as hereditary multi ple exostosis. Osteochondromas of the spine are rarely encountered in clinical practice. These lesions are reported more commonly with neural compression in cases associated with hereditary multi ple exostosis. The authors describe a unusual clinical manifestation of a solitary osteochondroma located in the rig ht posterior arch of the atlas. Complete removal of the tumor was performed resulting in the relief of neck pain and spastic quadriparesis. Although unusual, osteochondromas of the cervical spine must be considered in patients with persistent neck pain and progressive symptoms of myelopathy. Computed tomography and magnetic resonance imaging in conjunction with plain radiograms is the neuroradiological modality of choice. The diagnosis and surgical excision of these tumors are important because they can cause spinal stenosis resulting in neural tissue compression and myelopathy. © 2012 by Korean Society of Spine Surgery.