Immune-mediated necrotizing myopathy (IMNM) is a class of idiopathic inflammatory myopathies. According to the types of antibodies in serum, IMNM can be divided into three subtypes: anti-signal recognition particle (SRP) necrotizing myopathy, 3-hydroxy-3-methylglutaryl-coenzyme A reductase (HMGCR) necrotizing myopathy, and serum antibody-negative necrotizing myopathy. Different subtypes of IMNM have common characteristics, but the specific pathological mechanisms differ. Anti-SRP necrotizing myopathy is an important type of IMNM. At present, the pathogenesis of the disease is unclear. Most views suggest that the disease is mainly caused by an autoimmune response; therefore, the therapeutic strategy is primarily immune regulation. Recent studies have implicated non-immune mechanisms such as endoplasmic reticulum stress and autophagy in the occurrence and development of the disease. Here, we review what is known about the pathogenesis of anti-SRP necrotizing myopathy and summarize the latest research progress, aiming to better understand the disease and provide new ideas for treatment targets.