Background. Autoimmune limbic encephalitis in children occurs most frequently in those with antibodies against the N-methyl-D-aspartate glutamatergic receptor. We report the case of a 14-year-old girl who was diagnosed with antileucine-rich glioma-inactivated protein 1 limbic encephalitis.

Case. A fourteen years old, previously healthy girl applied to the emergency department with suspicion of dystonic seizure, ataxia, gait disturbance and speech disorders. Serum sample of the patient was positive for leucine-rich glioma inactivated protein 1 IgG.

Conclusions. Although it is a rare disease in childhood, in the presence of new onset psychotic symptoms or altered mental state, concomittant hyponatremia and unique type of seizures, anti leucine-rich glioma inactivated protein 1encephalitis should be considered in differential diagnosis.

Keywords: autoimmune encephalitis, faciobrachial dystonic seizure, leucine-rich glioma-inactivated 1 encephalitis, pediatric