Objective To discuss the key points of anesthesia management for children with congenital portosystemic shunts (CPSS) undergoing surgical treatment, in order to provide reference for future work. Methods A total of 23 children with CPSS who underwent surgical treatment in Shanghai Children's Medical Center, School of Medicine, Shanghai Jiao Tong University from May 2014 to March 2022 were selected and their clinical data were retrospectively analyzed. The characteristics of perioperative anesthesia management were summarized. Results Among these 23 patients, the main preoperative complications were pulmonary hypertension (PH), pulmonary arteriovenous fistula, hepatopulmonary syndrome (HPS). Their American Society of Anesthesiologists (ASA) scores were from Ⅱ to Ⅳ. All the patients were treated with combined intravenous and inhaled general anesthesia under mechanical ventilation. During the operation, four patients were given vasoactive agents, two patients were given pulmonary vasodilators, nine patients were given blood products and six patients were given coagulants. After surgery, one patient was admitted to the intensive care unit with endotracheal intubation, while the remaining 22 patients were sent to the post anesthesia care unit after endotracheal extubation. Conclusions For perioperative anesthesia management of children with CPSS, much attention should be drawn to the pathophysiological changes of primary diseases, while the challenges and risks of anesthesia management due to related complications (such as PH and HPS) are also of great concern.