This study aimed to examine the epidemiology and clinical characteristics of cerebral alveolar echinococcosis in the Tibetan region of Sichuan, China. A retrospective analysis of hospitalized cases of cerebral alveolar echinococcosis from six 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. Of 119 cerebral alveolar echinococcosis patients, 76 were male and 43 were female. Occupationally, 62 were farmers, 46 were herdsmen, nine were monks, and two were students. The mean age was 43.9 (± 13.9) years. The primary clinical manifestations were dizziness, headaches, and epilepsy. The incidence of cerebral alveolar echinococcosis 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, and the lesion had multiple aggregated small vesicular structures as its unique imaging feature. Among 98 follow-up cases, 62 could live independently (63.3%); 18 deaths were recorded (18.4%), with an approximate 5-year survival rate of 81.6%. Regular examination of patients with first diagnosis of hepatic alveolar echinococcosis without a combination of echinococcosis in other parts of the body can help monitor and prevent the occurrence of cerebral alveolar echinococcosis, improve the understanding of cerebral alveolar echinococcosis in Tibetan areas of Sichuan.