Dermatomyositis (DM) complicated by rapidly progressive interstitial lung disease (RP-ILD) is a type of connective tissue disease (CTD) with rapid disease progression and high mortality. Traditional treatment regimens such as glucocorticoids, cyclophosphamide, and calcineurin inhibitors are available, and some of them are not effective. Tocilizumab is an IL-6 receptor (IL-6R) antagonist that is widely used to improve the symptoms and signs of autoimmune diseases as a commonly used drug in the treatment of connective tissue diseases such as rheumatoid arthritis and Takayasu's arteritis. Related studies have shown that it has a certain efficacy in alleviating the acute response of connective tissue disease-associated interstitial lung disease (CTD-ILD). This may be related to the increased levels of IL-6, KL-6, and serum ferritin in ILD. In this paper, we report two cases of successful treatment of dermatomyositis complicated with interstitial lung disease with tocilizumab, and review the literature to explore the diagnosis and treatment experience of DM ILD and further explore its potential mechanism.