We describe a 4-year-old girl with hemifacial atrophy. She had a linear white-colored sclerotic plaque on the right submandibular area of skin. Histologic findings of the lesion were consistent with a diagnosis of scleroderma. The relationship between progressive facial hemiatrophy and linear scleroderma are discussed. We think that linear scleroderma of childhood and hemifacial atrophy have considerable clinical overlap and these two syndromes appear to be manifestations of the same or related pathogenic processes. Recently, the beneficial effects of 1.25-dihydroxyvitamin D-3 (calcitriol) were reported in adults and in children with linear scleroderma. We assessed the efficacy of oral calcitriol treatment in our patient. Our result indicates that calcitriol may be an effective agent for treating localized scleroderma in children.