Dermatomyositis is a rare autoimmune disorder in which an abnormal immune reaction against vascular endothelial antigens and endomysium leads to obstructive inflammatory changes of blood vessels within muscles, skin and other tissues. The disease is characterized by involvement of muscles, and less frequently of other systems, including the gastrointestinal tract, heart and lungs. Dermatomyositis may be diagnosed based on a detailed patient history, through clinical examination, detection of characteristic physical findings and certain specialized tests. Additional imaging studies may be performed to aid in the diagnosis. These include magnetic resonance imaging and ultrasound of the affected muscles. Magnetic resonance imaging is the modality of choice in the diagnostic work-up and monitoring of dermatomyositis affecting muscles, fasciae, and the subcutis. It may recognize acute inflammatory edematous changes in the affected muscles as well fatty replacement and atrophy. The role of ultrasound to diagnose and follow up muscle echogenicity, vascularity, elasticity and volume during treatment has increased over the last years in both adults and children. Ultrasound is used to discriminate between high and low disease activity, may show features of subclinical disease and may be used to confirm remission.