We report a patient with linear scleroderma en coup de sabre without neurological symptoms despite the presence of large white matter lesions. The patient underwent 3.0-T magnetic resonance (MR) examinations including diffusion tensor imaging, time-resolved contrast-enhanced MR angiography, susceptibility-weighted imaging, and proton MR spectroscopy. These imaging findings suggested increased vascular permeability and microbleeding without abnormalities of metabolites. Our observation is consistent with vasculopathy and may be helpful in the proper diagnosis and treatment of linear scleroderma en coup de sabre.