BACKGROUND: Autoimmune encephalitis can result in significant neurological and psychiatric morbidity and mortality in patients of all ages and often does not respond to standard therapies. Recent reports suggest efficacy of tocilizumab, a monoclonal antibody against interleukin 6, in refractory autoimmune encephalitis. RESULTS: We describe three children with refractory autoimmune encephalitis who experienced a robust, immediate clinical response following treatment with tocilizumab. CONCLUSION: These findings support the efficacy and short-term safety of tocilizumab as a third-line treatment for refractory autoimmune encephalitis in children.