We present the unusual case of a patient with an aquaporin 4 antibody-seropositive neuromyelitis optica spectrum disorder who presented with autonomic dysregulation, cognitive impairment, and symptoms of psychosis. Only a few previous cases have been described with similar psychiatric symptoms. Brain MRI showed an abnormal hyperintense T2 signal of the hypothalamus and, to a lesser extent, a minor hyperintense signal of the right optic nerve. Her symptoms and MR abnormalities improved after high-dose methylprednisolone.