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Juvenile localized scleroderma (JLS) is a rare autoimmune disease in children. The lack of specificity in the early symptoms of JLS makes it easy to be misdiagnosed and missed, which leads to the continuous progression of the lesions, and even causes systemic malfunction and growth disorder. For identifying the etiology of JLS and guiding the development and application of effective therapeutic drugs, we reviewed recent theories about the pathogenesis of JLS and summarized the traditional treatment options and relevant new therapeutic drugs, aiming to further enhance the fundamental understanding of JLS and its therapeutic progress.