A 5-month-old female Rottweiler dog was diagnosed to have a neurodegenerative disease that has been recently reported in Rottweilers from North America and Europe. The dog was presented with progressive signs of ataxia, tetraparesis and inspiratory stridor. The clinical investigation included analysis of CSF, radiography, myelography and electrophysiological testing. No evidence of vertebral malformation or inflammatory CNS disease was identified. Bilateral laryngeal paralysis was identified in the lightly anaesthetised dog. Electromyography showed abnormal spontaneous activity from the intrinsic musculature of the larynx. At necropsy there were no gross abnormalities of the nervous system but there was atrophy of the dorsal cricoarytenoid muscles of the larynx. There were widespread histological abnormalities throughout the nervous system including neuronal vacuolation, spongiform changes in the neuropil and axonal degeneration which was most prominent in the spinal cord. These clinical and pathological findings are consistent with the diagnosis of a new neurodegenerative disease reported from North America and Europe. This diagnosis is of particular significance in Australia where transmissible spongiform encephalopathies have not been identified.