This article reviews the current status of the classification and treatment of the juvenile idiopathic inflammatory myopathies. The intent of classification is to define homogeneous groups that share similar clinical features, disease courses, and responses to therapy. The classification scheme proposed includes clinicopathologic subsets, serologic subjects based on the presence of myositis-specific and myositis-associated autoantibodies, and environmental triggers of myositis. Juvenile dermatomyositis is the most common and widely recognized of these disorders. The second part reviews the history of treatment of juvenile dermatomyositis and discusses agents to consider for patients with refractory disease, unacceptable steroid toxicity, or poor prognostic factors.