Anti- HMGR antibody positive immune mediated necrotizing myopathy is a rare disease was first recognized and characterised in patients with a history of stating exposure and immune -mediated necrotizing myopathy. We reported a rare case of patient presenting with features of proximal myopathy which has a wide differential diagnoses of which a diagnosis of anti- HMGCR-antibody associated myopathy should not be neglected. Effective treatment can Improve the prognosis if diagnosed in time, We provide a summary of clinical findings, pathological features, muscle imaging and immunogenetic risk factors of the disease. We also discuss treatment strategies and approaches to monitoring the therapeutic response. lastly, we briefly summaries the current understanding of the pathophysiology of the disease and postulate a mode for autoimmunity initiation and propagation in the disease.