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A 44-year-old man was admitted to the hospital for generalized sclerotic and atrophic patches with itching on the trunk and extremities, which had exacerbated progressively for 8 years. Physical examination revealed hyperpigmentation and hypopigmentation in lesions with leather-like stiffness. No lesions were found in oral mucosa or anogenital area, and no systemic involvement or abnormity was found in routine laboratory examination. Histopathological examination showed hyperkeratosis, atrophy and thinning of epidermis, liquifaction degeneration of basal cells, as well as a band-like homogeneous degeneration in papillary layer of dermis. Proliferated, dense, and gently hyalinized collagen fibers strongly positive for eosin were observed in medium and lower dermis. The case was diagnosed as lichen sclerosus et atrophicus with localized scleroderma.