Voltage‑gated proton channel (Hv1) is a special cation channel that mainly participates in the transport of hydrogen ions out of cells and assists in the production of reactive oxygen species in microglia. The Hv1 is mainly expressed in epithelial cells and immune cells of mammals, and plays an important role in central nervous system diseases and tumor diseases. This review introduces the molecular composition and distribution of Hv1, and summarizes the roles and related mechanisms of Hv1 in various neurological diseases such as traumatic spinal cord injury, stroke, multiple sclerosis, and Parkinson disease, which provides a new target and approach for the prevention and treatment of neurological diseases.