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.