The long non-coding RNA (lncRNA) existing in the human genome has attracted much attention because of its important regulatory role. More and more studies indicated that lncRNAs play significant roles in neurodevelopment, neuroplasticity and central nervous system diseases. lncRNA nuclear enriched abundant transcript 1 (NEAT1) is abnormally expressed in the central nervous system diseases such as Alzheimer’s disease (AD), Parkinson’s disease (PD), Huntington’s disease (HD) and amyotrophic lateral sclerosis (ALS), and is involved in important pathophysiological process. This article reviews the research progress of the NEAT1 in central nervous system diseases.