This study was designed to describe clinical features and course of a child with anti-N-methyl D-aspartate receptor (NMDA) receptor encephalitis. We present a 7-year-old girl with tumor-negative anti-NMDA receptor encephalitis. A previously healthy 7-year-old girl presented with two generalized tonic clonic seizures. She underwent an extensive workup, including cranial MRI and EEG. All results were unremarkable. She was diagnosed with epilepsy and discharged with phenitoin. She was readmitted with complaints of new seizures, choreodystonic movements, oromotor dyskinesia and dysarthria 3 days after the discharge. CSF analysis showed a mild pleocytosis. Acyclovir, cefotaxime, and clarithromycin were administered empirically. Repeated cranial MRI was normal. Video EEG monitoring indicated focal seizures originating from the temporal region of the left hemisphere. The patient required midazolam anesthesia for nearly 15 days. IVIG was started on day 2 of second admission. Carbamazepine, valproic acid, topiramate and clobazam were added respectively. Pulse methylprednisolone treatment was started on day 7 of second admission for 3 days continued with oral prednisone. Several days afterward, she became aphasic and seizures were continued. Therefore she then underwent a 7 course of plasma exchange. On day 18 of second admission, NMDA receptor antibody was reported as positive. Rituximab treatment was added weekly (375 mg/m 2 10 weeks). In addition, she took 1 mg/kg/day of prednisone for 5 months which was tapered and treatment continued with oral azathioprine and IVIG with 3 weeks interval. Over the course of 8 months, seizures stopped completely. However she continues to be nonverbal and required maximal assistance in mobility. Recovery from Anti-NMDA receptor encephalitis is slow and the patients may have some disturbances in their motor function and cognition. The pathologic mechanism has been elucidated gradually but the optimal treatment has not yet been clarified. Therefore effective treatment strategies should be developed for intractable cases.