Interstitial lung disease (ILD) is common in patients with polymyositis (PM) and dermatomyositis (DM), and is a major cause of morbidity. Although its cause is unknown, it is known to be closely associated with autoimmune disorders. Its manifestation has been found to be quite heterogeneous, as demonstrated by the differences among PM/DM patients in their immunologic profiles and histopathologic findings, which suggest variations in immunopathogenetic mechanisms. We review the clinicopathologic and immunologic findings in ILD associated with PM/DM, and discuss recent advances in classification, autoantibodies, and treatment. The most critical issues are to clarify the immunopathogenesis of severe forms of ILD, such as rapidly progressive ILD associated with amyopathic DM, and to establish the most appropriate therapy.