A 39 years old woman was admitted to our hospital with a status epilepticus, with high fever of 41°C. Magnetic resonance Imaging (MRI) revealed high signal intensities of both sides of thalami and hypothalami in T2 weighted and fluid attenuated inversion recovery (FLAIR) images. A needle biopsy of the thalamic lesion was consistent with neuromyelitis optica spectrum disorder although her serum antibody to aquaporin-4 was negative. The level of orexin in celebrospinal fluid (CSF) was reduced. She presented hypersomnia, which didn’t improve even after intravenous methylprednisolone 1 g daily for 3 days. Administration of oral modafinil extended her waking time. There is a number of reports about neuromyelitis optica (NMO) with hypothalamic lesions. We report this case as important suggestion of treatment of thse cases.