The clinical characteristics, laboratory tests, treatment and prognostic characteristics of a case of neurexin-3α antibody-mediated autoimmune encephalitis,who was admitted in Xi′an International Medical Center Hospital on January 4, 2020 were summaried. The patient was a 56-year-old male with pre-symptoms of infection before his illness. The main manifestations were rapid progress of cognitive dysfunction, mental and behavioral abnormalities, refractory epilepsy, and status epilepticus, dystonia, accompanied by autonomic nerve involvement such as increased heart rate, breathing, and sweating, suspected of central hypoventilation, clinical manifestations were similar to N-methyl-D-aspartate-receptor (NMDAR) encephalitis and magnetic resonance imaging suggested bilateral hippocampal, temporal, and insular high signals, cerebrospinal fluid examination and other laboratory tests were normal. After treatment with methylprednisolone and intravenous immunogloblin, he died a few days after being discharged. The clinical manifestations of neurexin-3α antibody-mediated autoimmune encephalitis were similar to NMDAR encephalitis and severe, and neurexin-3α antibodies need to be further tested to confirm the diagnosis when NMDAR antibody was negative.