JUNIPER PUBLISHERS-OPEN ACCESS JOURNAL OF HEAD NECK & SPINE SURGERY Abstract Chiari III malformation, one of the rare variants of Chiari malformations, is including a small dysplastic posterior fossa, hydrocephalus, medullary abnormalities, and hindbrain herniation into a low occipital/high cervical encephalocele. This type can be lethal if not treated and is related to severe neurological deficits, so surgical care should immediately be undertaken. We are presenting a 1.5-month-old male infant with Chiari III malformation that was managed surgically with good outcome in addition, review the radiological, clinical and pathogenesis of Chiari III malformation. Introduction Type III Chiari malformation is a very rare condition that is described by Chiari in 1891.This type of Chiari defined as hindbrain herniation into a high cervical encephalocele or low occipital, and osseous defects with features of type II Chiari malformation (including a small posterior fo
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