ABSTRACT.- In Brazil, CNS diseases in equids were little known until the 1980s. Since then, several diagnostic laboratories have been operating in different universities, initially in the South and Southeast regions and, later, in the Central-West, Northeast and North regions. However, the knowledge accumulated from the diagnoses of nervous system diseases of equids made at these institutions over the years has not been reviewed comprehensively, and many papers are published in Portuguese. Here, we review 18 diseases that occur in the nervous system of equids in Brazil, including some critical infectious diseases (rabies, equine encephalomyelitides, equine herpesviral myeloencephalopathy, protozoan myeloencephalitis), and diseases caused by toxic plants and mycotoxins. Our review provides reference data to assist pathologists and clinicians in diagnosing neurologic diseases in equids. We also hope it will serve as a reference for professionals in this field abroad, allowing them to compare data in the epidemiology and pathology of the nervous system diseases of equids in different regions of the world. © 2025 The Author(s)