We herein report a case of immune-mediated necrotizing myopathy (IMNM) in a patient with microscopic polyangiitis (MPA). A 77-year-old Japanese woman presented with a 2-day history of proximal muscle weakness and myalgia, with elevated serum creatinine kinase (CK) levels. Findings of a muscle biopsy were compatible with IMNM; however, anti-SRP and anti-HMGCR antibodies were negative. She also had peripheral neuropathy with elevated serum MPO-ANCA titers, leading to a diagnosis of MPA. IMNM can be a pathological result of MPA muscle involvement.