Juvenile dermatomyositis (JDM) is a rare, multisystemic, idiopathic vasculopathy mainly affecting the muscles and the skin. Gastrointestinal system, lungs, joints and heart may also be involved. Characteristic skin findings are heliotrope rash and Gottron papules but extensive skin involvement as large necrotic lesions are rarely reported. Calcinosis is one of the major issues in the long term. Delay in diagnosis, inadequate therapy at the initial phase, prolonged persistent disease activity are considered as major risk factors for the development of calcinosis. Treatment of calcinosis is also a major issue because no single treatment modality has been found to reproducibly stop or reverse calcification.