A 9-year-old girl with linear scleroderma en coup de sabre (LSCS) developed progressive white matter involvement, presenting as intractable hemiplegic migraine-like attacks induced by exercise. Clinical and radiological stabilization was achieved under immunosuppressant treatment, with evidence suggesting a chronic inflammatory process of the central nervous system. The study discusses possible immunopathogenic mechanisms responsible for neurocutaneous involvement in LSCS, supporting the hypothesis of an autoimmune and inflammatory vasculopathy. The occurrence of hemiplegic migraine triggered by exertion provides new insights into the pathogenesis of scleroderma disorder, emphasizing the importance of intensive immunosuppression in selected cases.