As a kind of autoimmune encephalitis which was just identified, the clinical manifestations of the anti-N methyl-D aspartate (anti-NMDA) receptor encephalitis are complex, diverse and in severe condition. The immunotherapy has shown good effect on the treatment but in generally, the diagnosis and treatment are still in the experience accumulation stage. More clinical research in different population is necessary, for example, in the Chinese population. This study was completed in anti-NMDA receptor encephalitis patients who were diagnosed in Beijing Xuan Wu Hospital (China) during the time from 2011 to 2013. Total 33 patients were involved with the average age of 29.7 years old when the diseases were onset. With diverse clinical manifestations, most patients displayed positively by NMDAR antibody test and 63.6% of them were associated with elevated CSF-IgA. Patients also showed abnormal MRI and EEG. Only three patients had teratomas. With hormone therapy, gamma globulin treatment or plasma exchange, more than three quarters of patients fully recovered and the others had moderate symptoms. Based on our results, we suggest that NMDAR antibody test would be helpful to make a timely diagnosis and to administer immunotherapy.