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Limbic encephalitis (LE) with antibodies against leucine-rich glioma inactivated protein 1 (LGI1) is an autoimmune disease with variable clinical features, including seizures, cognitive disorders, psychiatric disturbances, and hyponatremia. The majority of these patients present faciobrachial dystonic seizures (FBDS), which are regarded as a characteristic symptom. A few cases have reported pilomotor seizures as the main manifestation of anti-LGI1 encephalitis. Here, we described a Chinese woman with frequent pilomotor seizures who was finally diagnosed as having anti-LGI1 encephalitis. Our report emphasizes the possible significance of pilomotor seizures in anti-LGI1 encephalitis.