We present the case of a 58-year-old female presenting with acute onset headache and decrease in left-sided facial sensation. The patient's background included diabetes Type 2, hypertension, migraine, anxiety with depression and scleroderma in her childhood. Imaging revealed foci of right frontal calcification and confluent white matter changes, reported as sequelae of a previous ischaemic episode. Following a second presentation with the same symptoms, further imaging showed a linear soft tissue scar overlying these changes, which suggested a diagnosis of linear scleroderma (en coup de sabre). On questioning, it was found that this had developed during the patient's late teens but had not progressed since that time. Coup de sabre type linear scleroderma is often associated with intracranial imaging findings, even in the absence of symptoms. The pathogenesis of neurological symptoms is poorly understood but does seem to respond to immunosuppression.