Background Juvenile dermatomyositis (JDM) is a chronic autoimmune disease characteristic by inflammation of small vessels within the skin,muscle and vital organs.But the clinical features and treatment of JDM have not been fully clarified.Data sources Databases underwent through PubMed for articles about the clinical features,myositis-specific antibodiesof JDM and its treatment,and we selected publications written in English which were relevant to the topic of this review.Results Clinical features and myositis-specific antibodies may predict the severity and prognosis of disease.Although the mortality rate has been lower with traditional treatments,such as corticosteroid,intravenous immunoglobulin,and diseasemodifying anti-rheumatic drugs such as methotrexate,their usages are variable.Novel biological therapies seem to be effective for refractory JDM patients,but more clinical trials are necessary.Conclusions JDM is a sever disease of childhood.We need to better understand recent advances of JDM in the context of clinical features including skin manifestations,muscle weakness and organ damage,myositis-specific antibodies and their associated outcomes and the treatment of disease.