脊髓性肌萎缩（spinal muscular atrophy, SMA）是一种罕见的神经肌肉性疾病，是儿童期最常见的遗传致死性疾病之一。这类患儿因为全身肌肉萎缩及其他畸形的存在，麻醉存在一定的难度。文章介绍该疾病的发病机制和分型、麻醉管理（麻醉前评估、麻醉方式和药物的选择、术后监护及镇痛）及其治疗进展对麻醉的影响，为拟行手术的SMA患儿的围手术期管理提供参考。“,”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.