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The term new onset refractory status epilepticus (NORSE) is used for patients who do not have a history of seizures and a structural disorder and in whom no etiology can be found. Its treatment is very difficult and its mortality is high. In this article, we present a 62-year-old patient whose etiology was not elucidated despite all examinations and who was diagnosed as having NORSE. Full clinical improvement was achieved with corticosteroid treatment.