A retrospective analysis was performed for 14 patients who were diagnosed with immune-mediated necrotizing myopathy in our hospital from January 1， 2017 to June 30， 2020 to summarize and analyze their clinical features and the features of muscle pathology and myositis-specific auto antibodies. Most of the 14 patients had disease onset in their middle age， and most of them were women， with an acute or sub acute course of the disease. Main clinical manifestations included muscle weakness （involving the proximal muscles of limbs， neck flexors， masticatory muscles）， and involvement of multiple systems， such as skin rash， oral ulcers， dry mouth， dry eyes， and interstitial lung disease. There was a moderate-to-severe elevation of creatine kinase， and electromyography showed myogenic damage. Detection of myositis-specific auto antibodies showed mainly anti-signal-recognition particle auto antibodies and anti-hydroxy-3-methyl-glutaryl-coa reductase auto antibodies. Muscle pathology showed diffuse or scattered necrosis， phagocytosis， and regeneration of muscle fibers， without or with a small amount of inflammatory cell infiltration. Immunohistochemical staining showed mainlythe infiltration of CD68⁺ macrophages. Immune-mediated necrotizing myopathyhas complex clinical manifestations involving multiple systems， which requires multidisciplinary diagnosis and treatment. In addition to routine auxiliary examinations， detection of myositis-specific auto antibodies is very important and can help with the diagnosis of this disease.