Dermatomyositis (DM) is a rare chronic autoimmune condition characterized by proximal muscle weakness, characteristic skin lesions and frequently, specific autoantibodies. Involvement of other organ systems, particularly the lungs, is usual and the condition is associated with malignancy in a significant proportion. The morbidity and mortality associated with DM remains high, despite the availability of a large number of therapeutic agents. Over the recent years, considerable progress has been made in the diagnosis and classification of these patients, particularly in the area of myositis-specific antibodies, which has provided further insight into the etiopathogenesis of this complex disease. Advances made in imaging techniques, especially MRI, have enhanced the diagnostic pathway in DM and provided novel means of monitoring disease activity and response to treatment. Although a number of exciting therapeutic trials are underway, the evidence base for the treatment of DM is found wanting. The aim of this review is to give an update on the approach to management of DM.