<ns3:p><ns3:bold>Introduction:</ns3:bold> Linear scleroderma <ns3:italic>en coup de sabre</ns3:italic> is a subtype of scleroderma hallmarked by cutaneous and extracutaneous manifestations in which neurological symptoms can be a predominant feature of this condition.</ns3:p><ns3:p> </ns3:p><ns3:p> <ns3:bold>Case presentation:</ns3:bold> We report a case of a previously healthy 47-year-old male who developed neuropsychiatric symptoms and right-sided cephalalgia for two months. Clinical examination revealed a right frontoparietal cutaneous lesion and neurological findings suggesting a medial prefrontal syndrome. The neuroimaging evaluation identified scalp and bone thinning adjacent to the skin lesion and cortical-subcortical white matter hyperintensity due to vasogenic oedema at the right frontal and parietal region. A biopsy from the affected area revealed reactive gliosis.</ns3:p><ns3:p> </ns3:p><ns3:p> <ns3:bold>Conclusion:</ns3:bold> To our knowledge, this is the first linear scleroderma <ns3:italic>en coup de sabre</ns3:italic> report associated with a neurological involvement typical of a medial prefrontal syndrome. This case highlights the importance of clinical acuity in recognising atypical phenotypes within the spectrum of this uncommon disease.</ns3:p>