Chiari畸形Ⅲ型（CM-Ⅲ）属罕见亚型，在所有Chiari畸形分型中占比低于1%。该疾病常伴枕颈部脑膨出、脑干或后组脑神经功能障碍、脑积水及复杂静脉窦变异，围术期决策需同时处理困难气道、膨出神经组织保护、脑脊液循环重建等。目前尚无CM-Ⅲ统一规范化诊疗方案，临床需结合膨出囊内容物、静脉解剖结构、脑积水情况及全身合并畸形制定个体化精准治疗策略。本文基于近年影像分型、病例系列及多学科管理证据，提出以产前识别与分娩规划、影像风险分层、个体化手术时间窗、脑积水分层处置、严密硬膜修补和早期连续康复为核心的管理框架，旨在为规范化治疗CM-Ⅲ提供可操作参考。对于膨出囊破裂或脑脊液漏、进行性呼吸衰竭或急性颅内压增高的患儿，应在复苏稳定的同时实施急诊干预；病情稳定者宜完成多学科诊疗评估后，于新生儿早期行手术治疗。

Chiari malformation type Ⅲ (CM-Ⅲ) is a rare subtype, accounting for less than 1% of all Chiari malformation subtypes. CM-Ⅲ is frequently complicated by occipitocervical encephalocele, brainstem or lower cranial nerve dysfunction, hydrocephalus and complex venous sinus variations. Perioperative decisions therefore need to address the difficult airway, protection of the encephalocele, restoration of cerebrospinal fluid circulation, and long-term neurodevelopment. At present, there are no unified standardized diagnostic and therapeutic protocols for CM-Ⅲ. Individualized precise therapeutic strategies should be formulated according to the contents of the herniated sac, venous anatomical features, status of hydrocephalus and systemic associated malformations. Based on recent imaging classifications, case series, and multidisciplinary management evidence, this review proposes a practical framework centered on prenatal recognition and delivery planning, imaging-based risk stratification, conditional surgical timing, stratified hydrocephalus management, watertight dural closure, and early continuous rehabilitation, aiming to provide practical references for standardized treatment of CM-Ⅲ. Emergency intervention should accompany resuscitation and stabilization in patients with sac rupture or cerebrospinal fluid leakage, progressive respiratory failure, or acute intracranial hypertension. For stable patients, surgery should be conducted in the early neonatal period after completing multidisciplinary evaluation.