Idiopathic inflammatory myopathies (IIM) are a group of heterogeneous, systemic autoimmune rheumatic diseases characterized by muscle inflammation and a varying degree of extra-muscular involvement, including cutaneous, pulmonary, vascular, gastrointestinal, cardiac, and joint involvement. This group includes dermatomyositis (DM), anti-synthetase syndrome (ASyS), immune-mediated necrotizing myopathy (IMNM), polymyositis (PM), inclusion body myositis (IBM), and myositis associated with other systemic autoimmune rheumatic diseases or malignancy [1,2]. Despite its rarity, the disease continues to evolve with new clinical and serological subtypes being identified over the years. Although glucocorticoids, conventional immunosuppressive therapies (including methotrexate, azathioprine, mycophenolate mofetil, cyclosporine, and tacrolimus), or immunomodulatory therapy (such as immunoglobulin therapy) are commonly used, many patients fail to achieve or sustain long-term low disease activity states or remission [3]. In this chapter, we review recent advancements in emerging therapeutic approaches for all sub-types of idiopathic inflammatory myopathies (IIM), excluding inclusion body myositis (IBM), with a focus on the mechanistic rationale supporting their use, their prospective impact on clinical outcomes across distinct IIM subtypes, and the barriers to broader clinical adoption (see Fig. 1). We also delineate future directions and highlight investigational agents with therapeutic promise.