A case of a mule foal with cerebellar Purkinje cell degeneration is described. A one-day-old mule foal was presented with lateral recumbency, obtunded mentation, poor metabolic status (general weakness, congestive mucous membranes, tachycardia, weak pulse, hypomotility and hypothermia) and cerebellar signs. After receiving medical treatment, the foal’s overall condition markedly improved but severe cerebellar ataxia and intention tremors were still present. Three weeks later, physical examination was normal but neurological signs had worsened and the foal was subjected to euthanasia. Postmortem findings revealed lesions consistent with corticocerebellar Purkinje cell degeneration. This is the first case report describing this condition in a mule.