Abstract: 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, leading to the continuous progression of the lesions, and even causing the body function damage and growth disorder. For identifying the etiology of JLS and guiding the development and application of effective therapeutic drugs, recent theories about the pathogenesis of JLS have been reviewed, as well as the traditional treatment options and relevant new therapeutic drugs have been summarized, aiming to further enhance the fundamental understanding of JLS and its therapeutic progress.