We report herein a caae of linear scleroderma resembling pseudoainhum. The patient was a 17-year-old boy who has 10 years history of linear, ivory colored, atraphic patch on his left 4th finger and left arm. The lesion was progreased slowly, and 2 years before he visited our hospital, his left 4th finger was amputated spontaneously. In histopathological examination, packed collagen, thick collagen bundle in dermis, and large areas of subcutaneous fat were replaced by eollagen fiber. Diagnosis was confirmed by the characteristics of clinical features and histopathologic findings of the skin. As therapeutic trials, steroid intralesional injection, immunotherapy with levamisole, agd penicillamine were attempted.