Detailed antibody examination revealed positive anti-melanoma differentiation-associated gene 5 antibodies.<h4>Conclusion</h4>We report the first case of anti-melanoma differentiation-associated gene 5 antibody-positive interstitial lung disease induced by enfortumab vedotin plus pembrolizumab in a patient with advanced urothelial carcinoma. The gene discussed is IFIH1; the disease is interstitial lung disease.