The idiopathic inflammatory myopathies are classified into four distinct subtypes: dermatomyositis, polymyositis, inclusion body myositis, and immunomediated necrotizing myopathy. These subtypes are clinically, histologically, and pathogenically distinct. Dermatomyositis, polymyositis, and necrotizing myopathy generally respond to immunotherapy, whereas inclusion body myositis does not. In this review, the authors focus on the clinical evaluation and management of idiopathic inflammatory myopathies and highlight recent therapeutic studies and the evolving role of myositis-specific antibodies.