Scleroderma is a rare chronic autoimmune connective tissue disease due to increased collagen production classified in to localized form and systemic form. Localized scleroderma also called ‘morphea’ are characterized by localized inflammation and fibrosis for the skin and the underlying tissue. En coupe de sabre is a rare craniofacial subtype of localized scleroderma. An 8-year-old female patient visited our hospital with linear white atrophic plaque extending from frontal scalp down to forehead for 5 months ago. There were no accompanying symptoms such as dysphagia, Raynaud’s phenomenon or dyspnea. A 3mm skin biopsy was performed from the lesion. Histologic examination showed thickening of the collagen with perivascular and periadnexal lymphocytic infiltration in dermis. Routine investigations including complete hemogram, urine routine, renal and liver function test, chest x-ray and electrocardiogram were within normal limits. However, specialized test like anti-nuclear antibody, anti-neutrophil cytoplasmic antibody and anti-scl-70 antibodies were positive. We diagnosed it as a linear scleroderma. We treated the facial lesion with triamcinolone injection and started to take d-penicillamine by rheumatology for preventing systemic involvement.