Introduction: Juvenile dermatomyositis ( JDM) with positive anti-melanoma differentiation-associated gene 5 (anti-MDA5) antibody have been associated with distinct clinical phenotype which includes characteristic mucocutaneous features, small hand joint arthritis, mucus membrane and skin ulcers, palmar papules, less muscle involvement and severe interstitial lung disease (ILD). Here, IFIH1 is linked to juvenile dermatomyositis.