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A postnatal cerebellar atazia of a type not previously reported in calves is described. Atazia was first noticed in affected animals when they were three to eight months of age, and was often accompanied by loss of the menace response in an animal which was not blind. The disorder was progressive. At postmortem cerebellar size was normal. Microscopic lesions consisted of primarily an apparent degeneration of cerebellar neurons without obvious evidence of inflammation. History, clinical signs, laboratory examination, gross pathology, histopathology, differential diagnosis, search for an etiologic agent and genetic histories are reported. It is concluded that this disorder may represent an hereditary cerebellar abiotrophy in Holstein cattle.