Children and adolescents with localized scleroderma (LS) are at high risk for extracutaneous-related functional impairment including hemiatrophy, arthropathy, seizures, and vision impairment. Compared with adult-onset LS, pediatric disease has a higher likelihood for poor outcome, with extracutaneous involvement twice as prevalent in linear scleroderma, disease relapses more common, and disease duration more than double. Consensus among pediatric rheumatologists on treating patients at risk for significant morbidity with systemic immunosuppressants has led to major improvements in outcome. This review discusses recent progress in assessment and treatment strategies and in our understanding of key disease pathways.