Mucopolysaccharidosis (MPS) is a congenital genetic disease. Patients often need surgery to relieve their symptoms and improve the quality of life. The changes in the general condition of MPS children, especially in the airway and heart, pose challenges to anesthesiologists. The paper summarizes the classification, pathophysiological changes and clinical manifestations of children with MPS. Furthermore, literature concerning preoperative evaluation, induction and maintenance of anesthesia, and key points about clinical anesthesia management was reviewed. Anesthesiologists need to comprehensively understand the patients' pathophysiological condition, and pay attention to preoperative evaluation and perioperative management to ensure smooth perioperative periods.