Dermatomyositis (DM) and polymyositis (PM) are rare idiopathic inflammatory myopathies. The severity of DM and PM is highly variable ranging from mild muscle weakness in cases of benign course to oesophagus, respiratory muscle and cardiac involvement in cases of extremely severe course. Most patients are treated with glucocorticoids or combined therapy of glucocorticoids with methotrexate and/or azathioprine. The presence of antibodies, duration of the disease before treatment beginning, severity of respiratory muscle weakness, dysphagia, cardiac involvement and presence of malignant disease are some of the predictors of outcome and therapy response. The aim of this paper is to present current knowledge about DM and PM treatment possibilities, treatment recommendations and novel therapies as an alternative option for patients who fail to respond to standard pharmacotherapy.