Linear scleroderma 'en coup de sabre' (LSCS) has been reported in association with intracranial abnormalities. We report the case of an 11-year-old boy with LSCS who presented with recurrent headaches. Cranial magnetic resonance imaging (MRI) and angiography were consistent with the diagnosis of a cerebral vasculitis. In addition, retinal examination showed an exudative inflammatory lesion consistent with vasculitis.
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