A 12-year-old intact female llama was euthanized following acute onset of spastic tetraparesis and recumbency with inability to rise. Postmortem examination revealed caudal cervical spinal cord compression due to a mass within the ventral spinal canal arising from the C6-C7 intervertebral disk space and attached to an irregularly thickened annulus fibrosis. On histopathologic examination, the mass was composed of amorphous acellular basophilic to amphophilic material admixed with irregularly arranged collagen bundles. The amorphous material was metachromatic and contained multiple small foci of markedly vacuolated round cells, characteristic of origin from the nucleus pulposus. Severe necrosis of all white matter tracts with astrocytic reaction was present in the overlying spinal cord segment. Ascending and descending Wallerian degeneration and dissecting interstitial astrogliosis were present within white matter tracts above and below the lesion, respectively. The diagnosis was compressive myelopathy due to chronic extrusion of the nucleus pulposus of the C6-C7 intervertebral disk. To the authors' knowledge, this is the first report of intervertebral disk disease in a camelid.