Statin-induced immune-mediated necrotizing myopathy, also known as anti-3-hydroxy-3-methylglutaryl-coenzyme A reductase (anti-HMGCR) myopathy, is an inflammatory myopathy that is triggered by statin exposure and persists after statin discontinuation. It is a rare side effect of statins, distinct from the more commonly recognized statin-induced myalgia, that is challenging to diagnose and treat. We describe a case of anti-HMGCR myopathy in a 59-year-old male with a prior history of statin intolerance presenting with markedly elevated creatinine kinase, myoglobinuria, and one month of progressive proximal muscle weakness after restarting atorvastatin 10 months prior to admission. High-dose glucocorticoids led to rapid clinical improvement, although the patient relapsed upon tapering. Remission was attained at three months after combination therapy with azathioprine, intravenous immunoglobulin, and a prolonged prednisone taper.