We report a case of anti-melanoma differentiation-associated gene 5 (MDA5) antibody-positive clinically amyopathic dermatomyositis (CADM) with rapidly progressive interstitial lung disease (RP-ILD), in which DAD was confirmed by transbronchial lung cryobiopsy at an early stage without respiratory failure. This evidence concerns the gene IFIH1 and retinitis pigmentosa 1.