Dermatomyositis is a form of idiopathic inflammatory myopathy that involves skeletal muscle and skin. The objectives of this review are to briefly describe the cutaneous manifestations of the disease, to raise some questions still debated about amyopathic dermatomyositis, and to reflect current knowledge of an interesting aspect in dermatomyositis as it is the risk to develop malignancy. Although clear evidence for a significant dermatomyositis-cancer association exists, optimal clinical or biological factors that predict an association with cancer have not been identified. Recently, some specific autoantibodies in dermatomyositis have been shown to be associated with internal malignancy. They open up the possibility to have available serological markers for detecting cancer-associated myositis in the near future.