Objective To summarize the key points of perioperative anesthesia management for children with Sturge‑Weber syndrome (SWS) who underwent hemispherotomy or hemispherectomy Methods Retrospective analysis was performed using medical records from SWS children who underwent hemispherotomy or hemispherectomy under general anesthesia from January 2017 to November 2020. Their demographic, perioperative and postoperative information were collected and analyzed. Results There were 8 SWS children who underwent such kind of surgery for refractory epilepsy, where 7 children (87.5%) manifested facial wine spots and 4 children (50.0%) were accompanied with glaucoma. No difficult airway caused by hemangioma was found. The intraoperative blood loss was at least 200 ml. Red blood cells were transfused in all children during operation, with a median volume of 260 (146.3‒357.5) ml. Because of severe bleeding, 4 children presented hypotension to various degrees, and one of them was accompanied with low blood oxygen saturation. No drugs were used to increase intraocular pressure during the perioperative period. After operation, 8 cases presented moderate or severe anemia, with a high intracranial infection rate (7/8, 87.5%). During the long‑term follow‑up, seizure outcome was evaluated and categorized according to Engel classification, and there were 7 cases of Engel grade I and 1 case of Engel grade Ⅱ. Moreover, no permanent dysfunction was found after operation. Conclusions Large‑volume hemorrhage is commonly seen during hemispherotomy or hemispherectomy in the treatment of refractory epilepsy caused by SWS. It is necessary to perform strict intraoperative monitoring and active perioperative management to maintain stable circulation. A large proportion of children are accompanied with glaucoma and drugs increasing intraocular pressure should be used cautiously during the perioperative period. Based on previous studies, it is necessary to fully evaluate whether the patient's airway is invaded by hemangioma before operation and to prepare well for difficult airway.