Spinal muscular atrophy (SMA) is the most common inherited lethal disease in childhood. The anesthetic management of these children is often difficult as a result of muscle weakness and other malformations. This paper briefly describes the pathogenesis and patterns, anesthetic management (including pre‑anesthetic assessment, anesthesia methods' and drugs' selection, post‑anesthetic monitor and analgesia) and the effect of recent therapy advances on anesthesia, to provide some ideas for anesthetists to manage the children of SMA who are undergoing surgery.