BackgroundAutoimmune encephalitis related to the leucine-rich glioma-inactivated protein 1(LGI1) antibody is the most prevalent in older adults, manifesting as seizures, faciobrachial dystonic seizures (FBDS), cognitive impairment, memory disturbance, hyponatremia and neuropsychiatric disorders. However the data pertaining to children affected by the disease is still limited.Case presentation and literature reviewsThis study presents a detailed report of a 6-year-old Chinese girl who experienced nose aches and faciobrachial dystonic seizures (FBDS). Electrolyte testing revealed that she had hyponatremia and brain MRI showed an abnormality in the left temporal pole. Additionally, anti-LGI1 antibodies were detected in both her serum (1:100) and CSF (1:30). The patient was treated with immunotherapy and symptom management, which proved effective. Furthermore, we provide a summary of 25 pediatric cases of anti-LGI1 encephalitis. Pediatric patients rarely exhibited FBDS and hyponatremia, and some cases presented with isolated syndromes. But the therapeutic outcomes of pediatric patients were generally good.ConclusionsIn this report, we describe a patient who developed a rare symptom of nose aches possibly as one of symptoms of anti-LGI1 encephalitis, which highlights the possibility of atypical symptoms in children that may be misdiagnosed. Reviewing the literature, the clinical features differed between pediatric and adult cases. Therefore, it is crucial to collect and analyze data from more cases to promote accurate diagnosis and timely treatment.