As with the treatment of many immune-mediated diseases, managing myositis encompasses diverse factors, which present a challenge to the physician caring for these patients. The idiopathic inflammatory myopathies (IIMs, also known as myositis), are fundamentally heterogeneous; many contributory immunological perturbations are involved in the pathogenesis of myositis, leading to varying clinical phenotypic presentations. Targeting any one or several of these deleterious pathways with a therapeutic agent might seem reasonable, but the desired response is not uniformly predictable. The presence of many serious extramuscular manifestations, such as severe skin rash, interstitial lung disease and arthritis, complicates the management of myositis. Myositis is rare, and very few large treatment trial results are available to guide clinicians. Outcome measures to effectively gauge treatment responses have been available for only a few years, and response criteria that incorporate critical core set measures continue to evolve. Nevertheless, a multitude of immunosuppressive and immunomodulatory agents are available to clinicians managing myositis, and the emergence of biologic agents targeting potential pathogenic pathways offers hope for mitigating or curing this enigmatic group of diseases. Paradigm shifts in the nonpharmacological approach to treat myositis have also occurred as more aggressive exercise regimens have shown benefit in patients, even those with active disease.