Scleroderma is a rare disease in children. Heterogeneous in its many forms, it ranges from circumscribed and self-limited pigmentary disorders to disabling and disfiguring involvement of an extremity. It also may include diffuse skin disease, multiple internal organ derangements, and a rapidly fatal outcome. Outcome varies widely and is related to the location and extent of cutaneous involvement, evaluation of disfigurement or impairment, and which treatment resources are available.