Background: Chiari type III is the rarest of the Chiari malformations, and it is usually associated with a dismal prognosis in terms of early death or severe disability in long-term survivors. Surgical treatment usually consists of primary closure of the occipital/cervical encephalocele, followed by delayed CSF shunting for the associated hydrocephalus.
Methods: In our case, we introduced a technical variant consisting in primary external drainage from the malformative sac, followed by closure of the encephalocele and, eventually, by CSF shunting.