With the development of social production, the progress of human civilization, and the growing aging population, neurodegenerative diseases are a global epidemic, a serious hazard to human health. Despite more comprehensive and in-depth understanding has been gained due to the long-term study of neurodegenerative diseases, its pathogenesis behind remains a mystery. It is estimated that about 98% of the transcriptional output of human genomes is RNA that does not encode protein, so called non-coding RNA (ncRNA), which has hidden activities in life and has a broad diversity of biological functions. MicroRNA is a class of small ncRNA, which has been intensively studied; long noncoding RNA (lncRNA) has recently received much attention, with a number of accumulated research results. The role of microRNAs and lncRNAs in the pathophysiological mechanism in several neurodegenerative diseases was reviewed.