We report a 54-year-old man with recurrent anti-melanoma differentiation-associated gene 5 (MDA5) antibody-positive dermatomyositis (DM)-associated interstitial lung disease (ILD) who was refractory to intensive triple therapy with high-dose glucocorticoids, tacrolimus, and intravenous cyclophosphamide. Here, IFIH1 is linked to dermatomyositis.