Tunneling nanotubes (TNTs) are F-actin-based thin channel-like structures connecting distant cells, which provide a new route for intercellular communication. Since TNTs are discovered, an increasing number of studies have demonstrated their roles in the transfer of diverse cargoes between connecting cells, including signaling molecules, RNAs, proteins, organelles, and even pathogens, which illustrate the diversity and complexity of TNTs' function. TNTs have been found in various types of cells, including neuronal cells. In the nervous system, the formation of TNTs between neurons or between neurons and astrocytes mediates electrical coupling and the transfer of pathogenic proteins associated with neurodegenerative diseases. Here, we summarized the current results of TNTs in the nervous system, including its formation, regulatory factors, functions, and potential benefits in the treatment of diseases.