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Localized scleroderma ��LS�� is clinically characterized by idiopathic fibrosis of localized skin and subcutaneous tissues. No reliable assessments of disease severity are available for LS. Several outcome measures have been proposed in recent publications, such as semiquantitative scoring system and computer-based scoring method. The performance of laser doppler flowmetry, super sound, and magnetic resonance have been explored in the measurement of disease severity of LS. Recent advances in therapy of this disease mainly focus on immunomodulators and anti-fibrosis agents. Some new treatment options for LS, such as tacrolimus, imiquimod, methotrexate, bosentan, phototherapy with UVA light, have been introduced and their efficacy has been estimated. This article reviews advances in disease severity measures and therapy of LS.