Respiratory system diseases, including infections, inflammation, fibrosis, cancer, and others, impose a substantial burden on human health worldwide. The respiratory tract is constantly exposed to external stimuli due to its connection with the outside environment. Therefore, the immune system plays a crucial role in respiratory diseases. Toll-like receptors (TLRs) recognize pathogens and initiate immune responses, serving as the first line of host defense against external pathogen invasion. Interleukin-1 receptor-associated kinases (IRAKs) are a group of kinases that mediate activation signals from TLRs and the interleukin-1 receptor (IL-1R). Among the four distinct IRAK family members, interleukin-1 receptor-associated kinase M (IRAK-M) uniquely functions as a pseudokinase and serves as a critical negative regulator of TLR/IL-1R signaling pathways, mediating diverse immunomodulatory effects in various pulmonary diseases. This review focuses on recent advancements in understanding the role of IRAK-M in lung disorders, aiming to provide a basis for future investigations into the pathogenesis and potential therapeutic targets for such conditions.