Respiratory diseases (e. g. , lung inflammation and pulmonary fibrosis) are a serious threat to human health. Mitochondria, organelles unique to eukaryotic cells, not only have important functions in energy production, biosynthesis, and the maintenance of intracellular homeostasis but also act as diverse signaling organelles involved in inflammation, proliferation, differentiation, cell repair, and other processes. The mitochondrial quality control system involves mitochondrial biogenesis, dynamics, and autophagy. Certain pathological mechanisms of respiratory diseases, such as oxidative stress and inflammation, are closely related to the dysregulation of mitochondrial quality control systems. This paper summarizes the progress of research into mitochondrial quality control dysregulation in respiratory diseases (chronic obstructive pulmonary disease, pulmonary fibrosis, acute lung injury, asthma, and bacterial pneumonia) to explore new ideas for the prevention and treatment of respiratory diseases.