Encephalocele is a broad term representing herniation of cranial contents through a congenital defect in the cranium. If only cerebrospinal fluid (CSF) and meninges herniate, it is termed as a meningocele. A menin goencephalocele is herniation of both neural elements and meninges. The incidence of encephalocele is 1 per 5000 live births. Anterior encephaloceles are more common in males while 70% of posterior or occipital encephaloceles occur in females. We present one case of a giant occipital encephalocele to highlight the problems encountered in its management. 

Keywords: Microcephaly; Occipital Encephalocele; Neurological Development.