Nucleotide binding oligomerization domain (NOD) and leucine-rich-repeat-containing protein X1 (NLRX1), a member of the NOD-like receptor (NLR) family, is an important regulator of the immune system. Studies have shown that NLRX1 could regulate nuclear factor kappa B signaling and typeⅠinterferon generation, regulate reactive oxygen species production, participate in autophagy, cell death and innate immunity response, and affect c-Jun N-terminal kinase and mitogen-activated protein kinase pathways. NLRX1 is closely related to the development and progression of respiratory virus infection, chronic obstructive pulmonary disease, obstructive sleep apnea hypopnea syndrome and lung tumors. Therefore, it is of great significance to understand the role and mechanism of NLRX1 in these diseases.