Abstract
Chiari malformation (CM) is a group of complex deformities of the posterior fossa and hindbrain, of which CMIII is the rarest. We report a term neonate, with an antenatal diagnosis of occipital encephalocele, who underwent resection of the encephalocele and ligation of vessels, with repair of a large scalp defect and dural reconstruction on day 4 of life. The parents of the child had been counselled for a guarded and poor prognosis on initial diagnosis. The child has had a good postoperative course without complications but suffers from cortical visual impairment and global developmental delay.