Abstract: 　Objective　To discusses the effectiveness of tocilizumab in the treatment of hypomyopathic dermatomysositis (HDM) combined with interstitial lung disease (ILD) in children. Methods　The clinical characteristic, treatment, and prognosis of HDM combined with ILD were analyzed in 2 patients. The related literatures were reviewed. Results　Both ten-year-old girl and 8-year-old boy had shortness of breath after activities, but had no clinical manifestations of muscle damage; both of them had typical rash, but had nornal muscle strength and  muscular tension. Laboratory tests showed the elevation of serum ferritin, lactate dehydrogenase, glutamate aminotransferase, and aspartate aminotransferase. Creatine kinase slightly increased in the initial test, and then was in the normal range in the following tests. The high resolution computed tomography showed that pulmonary interstitial lesions. HDM combined ILD was diagnosed clinically. The girl died after treatment with high-dose hormones, cyclophosphamide, cyclosporine, pirfenidone, and gamma globulin failed. The boy was stabled after conventional hormone treatment plus tocilizumab (240 mg twice). His laboratory indicators were in the normal range in the follow-up. Conclusions　The clinical manifestations and laboratory indicators aren't typical in childhood HDM. The mortality is high. Combined with tocilizumab treatment is effective in one case.