Objective:To summarize the epidemiology and clinical characteristics of cerebral alveolar echinococcosis (CAE) in the Tibetan region of Sichuan.Methods:A retrospective analysis of hospitalised cases of CAE from 6 medical units in the Garze Tibetan Autonomous Prefecture, Sichuan Province from January 2016 to June 2021 was conducted. The study focused on the characteristics, clinical presentation, and imaging features of the disease. Simple random sampling method was used to select equal number of cases of hepatic and cerebral blastomycosis to form the data sample, and the susceptibility factors of CAE were analyzed by Logistic regression.Results:Out of 119 CAE patients, 76 were male and 43 were female. Occupationally, 62 were farmers, 46 were herdsmen, 9 were monks, and 2 were students. The age was (43.9±13.9) years. The primary clinical manifestations were dizziness, headaches, and epilepsy. The incidence of CAE was most concentrated within 4 years after the diagnosis of hepatic alveolar echinococcosis (77/119, 64.7%). There were 86 cases (72.3%) with multiple intracranial echinococcosis lesions, with an average size of 2.0 cm×2.5 cm. The imaging features showed that the lesion was mainly concentrated in the anterior circulation blood supply area, surrounded by a wide edema band, which was significantly enhanced after enhanced scanning, and the lesion had multiple aggregated small vesicular structures as its unique imaging features. Among 98 follow-up cases, 62 could live independently (63.3%), with 18 deaths (18.4%), and an approximate five-year survival rate of 81.6%. The results of multiple regression analysis showed that susceptibility factors for CAE included female ( OR=2.742, 95% CI 1.039-7.236, P=0.042), consumption of raw meat/raw water ( OR=7.638, 95% CI 1.216-47.966, P=0.030), a history of hepatic encephalopathy ( OR=0.380, 95% CI 0.178-0.811, P=0.012), the presence of other parts of echinococcosis ( OR=2.968, 95% CI 1.113-7.910, P=0.030). Conclusions:CAE predominantly affects farmers and herdsmen, with a higher susceptibility among young and middle-aged individuals. Regular examination of patients with first diagnosis of hepatic alveolar echinococcosis without a combination of echinococcosis in other parts of the body can help to monitor and prevent the occurrence of CAE, improve the understanding of CAE in Tibetan areas of Sichuan, and strengthen the early diagnosis and prevention of CAE in all aspects.