Statin or 3-hydroxy-3-methylglutaryl-coenzyme A reductase (HMGCR) inhibitor is widely used and plays a vital role in the management of cardiovascular and cerebrovascular diseases. Statin is generally safe and its side effects are mostly mild and self-limiting. Immune-mediated necrotizing myositis (IMNM) is a rare and serious side effect characterized by the presence of anti-HMGCR inhibitor and myositis. Long-term immunosuppressive therapy is often required to manage it, and in refractory cases, the treatment can be very challenging. We report the case of a 55-year-old female with underlying diabetes mellitus and hyperlipidemia who developed refractory statin-induced IMNM despite being administered prednisolone, methotrexate, azathioprine, and immunoglobulin. After the introduction of rituximab, steroids were able to be tapered down to the lowest maintenance dose. Unfortunately, the patient subsequently succumbed to severe coronary artery disease (CAD) likely caused by the long-term steroid therapy, highlighting the difficulty and complications associated with the treatment of IMNM, especially in patients with cardiovascular risk factors.