Autophagy is an evolutionarily highly conservative lysosomal degradative process and closely associates with pathogenesis, process, treatment, drug resistance and relapse of acute lymphoblastic leukemia (ALL). Whether the autophagy displays resistance to chemotherapy or a tumor suppressor in ALL, it mainly depends on the context of autophagy in the cells. Understanding the different role of autophagy in different conditions for ALL, determing the autophagy signaling pathways and targeting combination with autophagy revulsants or inhibitors were significant for the therapy of ALL, particularly for the treatment of refractory/relapsed ALL patients. This review summarizes the role of autophagy in pathogenesis, developmant, drug resistance and treatment of ALL, providing some theoretical guidance of new drugs targeting autophagy for ALL therapy.