Abstract
Encephaloceles are cranial defects in which sac contains herniating brain, which is often gliotic. Congenitally, this defect may extend into posterior elements of cervical vertebrae and leads to occipitocervical encephalocele. When the size of this sac is larger than head size, they are termed as giant. Very young age and associated congenital anomalies in these patients pose significant challenges in diagnostic, anesthetic, and surgical techniques. We share a case of giant occipitocervical encephalocele managed at our institute and discuss about its management issues with review of literature.