Hydrogen sulfide (H₂S), an endogenous gas, succeeds after nitric oxide (NO) and carbon monoxide (CO) as the third gas signal molecule, neurotransmitter and cell protector. It plays an important role in many physiological processes of brain and surrounding tissues. Its role in the central nervous and cardiovascular system has been a focus of research in recent years. Its role has been partially proven in neurological diseases such as Alzheimer disease (AD), Parkinson disease (PD), cerebral ischemia, and febrile seizures (FS). This paper reviews the recent progress in the research of its role in those diseases, focusing on its neuroprotection and corresponding signal pathway, and its potential therapeutic value.