IntroductionTo identify the most effective treatment for juvenile dermatomyositis (JDM), considering efficacy, safety, impact on patients and improvement in their quality of life.Material and MethodsA systematic review was carried out comparing known treatments and immunobiological therapies, evaluating clinical improvement, adverse events and prognosis. The MEDLINE, PubMed, LILACS and Cochrane Library databases were used with children aged 0 to 18 diagnosed with JDM. The PRISMA 2020 statement was followed throughout the process.ResultsThe immunobiologics studied were rituximab (RTX) and anti-tumor necrosis factor drugs and used the Disease Activity Score to skin, Childhood Myositis Assessment Scale and Manual Muscle Testing tools. There was no difference in the response when RTX was used (early or late). The anti-TNF studies were carried out in a population that was refractory to the initial treatment and showed a significant improvement in muscle and skin disease activity.ConclusionsFor severe or refractory disease, biologics tend to be the medication with the best therapeutic response.