As a member of the nuclear receptor superfamily, pregnane X receptor (PXR) is a ligand-activated transcription factor. It is highly expressed in liver and intestine, as well as in kidney, brain and other organs. Since its discovery, PXR has been recognized as a sensor for exogenous and endogenous substances. In the activated state, it can regulate the expression of drug-metabolizing enzymes, transporters, and other metabo-lism-related proteins to exert detoxification effects. Its functions in the digestive system have been intensively explored due to its high expression in liver and intestine. In recent years, with the detection of PXR in more and more brain regions, its physiological and pathophysiological functions in the central nervous system have attracted much attention. This review summarized the expression profile of PXR in the central nervous sys-tem, its role in the blood-brain barrier, its relationship with neurological diseases and neurotoxicity, and its correlation with the behavioral science. The summary may provide guidance on clinical medication and new ideas for drug development, and also be beneficial for future research on PXR functions in the central ner-vous system.