The endoplasmic reticulum is an important organelle which is essential for maintaining the homeostasis and survival in the cell, while various adverse stimuli, such as endoplasmic reticulum stress caused by hypoxia, oxidative stress and viral infection, results in the aggregates of misfolded and unfolded internal protein in the lumen of the cytoplasm, and calcium ion balance disorders. Cells with endoplasmic reticulum stress restore the normal function of the endoplasmic reticulum to maintain cell survival by reducing the workload, increasing efficiency, and accelerating the degradation. If the adverse stimuli that cause endoplasmic reticulum stress persists or is too strong, the function of the endoplasmic reticulum will not return to normal through active regulation, and the cell will initiate an apoptotic program. Endoplasmic reticulum stress is widely involved in the pathogenesis of various diseases. In respiratory diseases, it can mediate cell adaptive survival, and also affect the expression of certain important proteins in the lung to induce inflammation and apoptosis. The effects of endoplasmic reticulum stress on respiratory diseases is summarized below.