Dermatomyositis is a specific type of inflammatory myopathy with characteristic cutaneous findings. Patients may have skin disease without clinically apparent muscle disease, but this disorder is best thought of as a systemic process. Therefore, all patients with dermatomyositis skin lesions need appropriate evaluation for muscle disease, esophageal dysfunction, cardiopulmonary disease, and potential internal malignancy. There are many therapies that have been used for patients with dermatomyositis, but most are based upon case series or expert opinion rather than meta-analyses or randomized, placebo-controlled trials. Even those therapies that have been subjected to randomized, blinded, placebo-controlled trials include a mixture of patients with idiopathic inflammatory myopathy and do not utilize a validated assessment tool for measuring cutaneous disease responses. In this review, we discuss the therapies available as well as the internal associations with dermatomyositis.