Mitochondria are essential organelles that execute and coordinate various metabolic processes in cells. Mitochondrial dysfunction significantly affects cellular health and leads to a variety of diseases. The maintenance of mitochondrial health depends on dynamic changes in the mitochondrial membrane network and effective mitochondrial quality control mechanisms. Increasing evidence suggests that mitochondria-associated endoplasmic reticulum membranes (MAMs), formed between mitochondria and the endoplasmic reticulum, play a crucial role in regulating mitochondrial quality control. This article reviews the research progress of MAMs in regulating mitochondrial quality control, revealing that the structural and functional changes of MAMs are closely related to disorders of mitochondrial quality control, providing new perspectives and potential therapeutic targets for understanding and treating associated diseases. Future studies need to further clarify the specific regulatory mechanisms of MAMs in different diseases and develop effective intervention strategies, with the aim of achieving precise treatment of associated diseases in clinical practice.