Juvenile Dermatomyositis is a very rare autoimmune disease with a subacute to chronic presentation. It affects patients under 16 years old, manifesting with proximal muscle weakness and characteristic skin lesions, it can be associated with autoimmune disorders and malignant neoplasms. The objective of the study is to present a case with its clinical characteristics, diagnostic process, and treatment, in order to achieve a timely diagnosis and avoid the associated morbidity and mortality. A 16-year-old female patient is presented with proximal muscle weakness, facial erythema, Gottron's papules, and asthenia. Treatment with methylprednisolone and methotrexate was started with subsequent remission. The therapeutic options should be evaluated individually, taking into account the severity criteria, in order to choose the best treatment to achieve remission and at the same time reducing the risk of adverse effects associated with treatment.