Dermatomyositis is a rare inflammatory myopathy with characteristic skin manifestations and muscular weakness. The disease can be categorized as adult idiopathic, juvenile, or amyopathic dermatomyositis as well as that associated with a connective tissue disease or a malignancy. Immunologic factors are most likely involved in the pathogenesis of the disease; however, genetic and environmental issues may also play important roles. Treatment with immunosuppressive agents has proved successful in the majority of patients, although significant morbidity still occurs.