Abstract: A 8-year-old boy was seen for indurated skin of right thigh, buttock and waist for 8 years. The lesions were unilateral with mild hypertrichosis and pigmentation. Skin histology showed hyperkeratosis of the epidermis, increased pigment in the basal layer, and thickened dense collagen fibers in the middle dermis with no obvious inflammatory infiltrates and no obvious atrophy of appendages. Alcian blue stainingwas positive. The diagnosis of segmental stiff skin syndrome was made. 

Key words: segmental stiff skin syndrome, linear scleroderma