ABSTRACTFive Boxer dogs with a new neurological disease are described. There is a progressive ataxia and weakness, initially in the hind‐legs but later involving forelegs. The onset of signs is usually around 6 months of age although an older dog was affected. Proprioceptive function, muscle tone and tendon reflexes are diminished or absent while pedal reflexes and pain sensation are preserved. Muscle atrophy is minimal. The peripheral nerves are depleted in myelinated fibres. Nerve roots and, to a lesser extent, peripheral nerves show demyelination/remyelination changes and in the cervical ventral roots in particular, regenerative clusters are present. Forelimb muscles show evidence of re‐innervation. In the CNS numerous swollen axons are present. The dorsolateral and ventral columns of the spinal cord are affected throughout their length. In the brain a number of nuclei and tracts, particularly in the lower brain stem, are affected and the superior olives are severely involved. The cerebral cortex and subcortical white matter appear normal. Various portions of the auditory and optic pathways are affected pathologically as are a number of cranial nerves. The axonal swellings contain a number of organelles including smooth endoplasmic reticulum, disordered neurofilaments, vesicles and mitochondria. The disease may be specific to Boxer dogs and may be inheri