Encephalocele is a form of neural tube defect, characterized by protrusion out of the meninges and brain tissue through a bony skull defect. As per the site of origin, these encephaloceles have been classified into different types, out of which the frontoethmoidal one is exclusively common in Southeast Asia with a reported incidence of 1 in 5,000. Neurological outcome of such malformations depends on the size of the sac, neural tissue content, hydrocephalus, associated infection, and other pathologies that accompany this condition. Here we describe the anaesthetic concerns and perioperative management of a giant occipital encephalocele. Copyright (C) 2013 S. Karger AG, Basel