To analyze the clinical data of autoimmune encephalitis with seizure as the first symptom. Methods: The clinical manifestations, laboratory test results, imaging examinations, electro-encephalogram (EEG) characteristics, treatment, and follow-up records of 8 autoimmune encephalitis cases with seizure as the first symptom were retrospectively reviewed and discussed in combination with literature. Results: Among the 8 cases, there were 5 cases with anti-NMDAR encephalitis, 2 cases with anti-GABAR encephalitis, and 1 case with anti-LGI1 encephalitis; there were 6 males and 2 females; the age of onset ranged from18 to 66 years old, with an average age of 50 years. All the patients experienced seizure as the first symptom and demonstrated slow response and short-term memory decline. Among them, 3 cases exhibited abnormal mental behavior, 1 exhibited speech disorder, and 1 exhibited refractory hyponatremia. All displayed seizure of one or more types. There were 6 cases with abnormal EEG and 4 cases with abnormal MRI. Conclusion: Early immunotherapy and anti-epileptic treatment are of great significance in controlling symptoms and improving prognosis for patients with autoimmune encephalitis with epilepsy as the first symptom.