Central nervous system (CNS) disorders, characterized by dysfunction and pathology in neurovascular units, represent a leading cause of disability, with high incidence and protracted courses. Tissue inhibitors of metalloproteinase 1 (TIMP-1) serve as natural inhibitors of matrix metalloproteinase 9 (MMP-9) and are critical for maintaining extracellular matrix homeostasis. Recent research has unveiled novel, MMP-independent functions of TIMP-1, as it acts as a multifaceted cell factor by binding to cell surface receptors. Fluctuations in TIMP-1 levels directly correlate with the occurrence and prognosis of CNS diseases, making TIMP-1 a promising target for promoting neurovascular unit repair. This review summarizes the recent research progress of TIMP-1 in CNS diseases, aiming to comprehensively explore its roles and mechanisms in the development of cerebrovascular injury, epilepsy, and neurodegenerative diseases.