En coup de sabre is a rare variation of linear scleroderma, which has a special appearance in the frontal or frontoparietal region. Although the etiology is not clearly known, it can be an autoimmune origin. This disease, which may be associated with neurological and ophthalmological findings, is more common in children. An 18-year-old female patient was admitted to our polyclinic with complaints of collapse and discoloration on the forehead line for approximately four years. The dermatologic examination revealed an atrophic, linear, and depressed lesion on the frontal region extending to the scalp. The patient was diagnosed with 'linear scleroderma (En coup de sabre)' with clinical and histopathological findings. No pathological findings were found on the ophthalmic examination of the patient. Neurological examination was normal and cranial magnetic resonance imaging revealed no pathological findings in the brain.