Circumscribed scleroderma (CS) shows a great variety of clinical pictures in children, but there is a preference of linear variants on the extremities and combined forms. Broad spectrum and high level of humoral immune phenomena, extension from distal to proximal, pansclerotic terminal state with defect of growth of the extremity are typical for linear variants on the extremities. Systemic forms of CS in farm of renal dysfunction, migraine-like attacks and arthralgia can exist. The consequences for treatment result from the immunopathogenesis. By immunosuppressive treatment it is possible to achieve standstill of the progression, softening of the lesions and partial involution of immune phenomena. The special aspects of CS in children will be exposed in 32 children of our case material.