Anti-N-methyl-D-aspartate receptor (anti-NMDAR) encephalitis is an auto-immune and paraneoplastic encephalitis which generally affects young adults. It is a multistage illness, with prominent extrapyramidal, neuropsychiatric and autonomic symptoms. The syndrome is frequently associated with an ovarian teratoma. Recently, it has become evident that anti-NMDAR encephalitis is more common in children and adolescents than was previously believed. Prognostic factors that determine a good outcome are presence of a tumour, prompt treatment and no need for admission to an intensive care unit. Increased awareness among paediatricians of this potentially life-threatening disease is important because early recognition and treatment will improve the patients' chances of a good clinical outcome. In this case report, we describe a 9-year-old girl with behavioural changes and severe extrapyramidal symptoms due to anti-NMDAR encephalitis associated with an ovarian teratoma. She was treated with a variety of immunomodulating therapies and made a slow, but good recovery.