Anti-NMDA receptor encephalitis is a severe disorder with a dramatic clinical presentation, often showing a purely initial neuropsychiatric phase, and evolving into a severe encephalopathy accompanied by involuntary movements, seizures and autonomic instability; the disease course is typically prolonged and needs intensive care treatment. We report the case of a 5-year-old child, diagnosed with Anti-NMDA receptor encephalitis with immune modulatory therapy.