To investigate the clinical features of Bickerstaff brainstem encephalitis (BBE) improving disease awareness and standards of diagnosis and treatment. Methods: Clinical data of 8 BBE patients were collected and retrospectively analyzed. Relevant literature was reviewed to summarize clinical features, pathogenesis, treatment, and prognosis of the disease. Results: All 8 patients showed acute onset. Before onset of BBE, 2 cases displayed upper respiratory infection, 2 cases diarrhea, and 1 case varicella zoster virus infection. In 3 cases, the initial symptom was numbness and weakness of the limbs with disorganized speech. There was 1 case each of unclear consciousness, weakness of both lower limbs, hoarseness with dysphagia, numbness of face and fingers, and dizziness with unsteady gait. The main clinical manifestations were as follows: acute ophthalmoplegia (extraocular n=7, intraocular n=1), ataxia (n=4), impaired consciousness (n= 4), deep tendon hyporeflexia or areflexia (n=5), positive pathology (n=8), facial nerve involvement (n=4), glossopharyngeal and vagus nerve involvement (n=6), and simultaneous involvement of cranial nerves (facial, glossopharyngeal, vagus, and hypoglossal nerves, n=3). Cerebrospinal fluid examination was normal in 2 patients, head MRI examination showed no characteristic manifestations in 4 patients, abnormal lesions in corresponding parts of the brainstem were found in 2 patients, and extensive dural enhancement was seen in 1 patient. All 5 cases that underwent routine serum GQ1b antibody testing were negative. All 8 patients were treated with immunoglobulin or combined glucocorticoid; 7 patients had a good prognosis, and 1 patient died. Conclusion: BBE mostly presents a single-phase benign course. Diagnosis is mainly based on clinical manifestations, and serum antibodies, cerebrospinal fluid pathogen genome, imaging, and neuroelectrophysiology serve in auxiliary diagnosis. Immunoglobulin or combined glucocorticoid therapy is effective treatment.