Dermatomyositis (DM) is an autoimmune disorder with idiopathic myopathy and characteristic skin manifestations that one often accompanied by an internal malignancy. The association between dermatomyositis and malignancy has been reported several times, although tonsillar carcinoma is extremely rare not only in far eastern populations but also in caucasian populations. We report two cases of Korean patients with dermatomyositis associated with tonsillar carcinoma.