Central nervous system diseases of horses represent difficult entities to diagnose. Clinical signs are frequently similar among different diseases and a complete neurological examination with the application of advanced diagnostic techniques can not be performed in horses. Knowledge of the neurological diseases by clinicians and collaboration between clinician and neuropathologist are critical to achieve a correct diagnosis. Accurate diagnosis is essential to give a prognosis and to control infectious diseases, including zoonoses. In this article, the main clinical and pathological features of equine central nervous system diseases reported in Italy are reviewed and classified according to the neuroanatomical localization, onset and progression of clinical signs.