Objective Due to the complexity of the etiology and pathogenesis, there is still no effective therapeutic strategies for diseases of the central nervous system (CNS). GABAB receptors are widely distributed in neurons and glial cells, and play important roles in the regulation of neurotransmitter release and neuronal excitability. It has been proven that GABAB receptors are involved in the development of a variety of CNS diseases, including epilepsy, depression, stroke, Alzheimer's disease, and autoimmune encephalitis. Therefore, clarifying the roles of GABAB receptors in the pathology of CNS diseases is essential for the development and clinical application of GABAB receptor-related drugs. In this paper, the specific mechanisms of GABAB receptors involved in various CNS diseases are reviewed based on the structures and functions of BABAB receptors.