Encephaloceles, also known as meningoencephaloceles, are a group of neurological disorders characterized by herniation of the cerebral parenchyma along with overlying meninges through openings in the skull bone. They can be seen following congenital defect in neural tube closure or acquired defect in the skull bone. We report a case of acquired frontoethmoidal encephalocele presenting with drug-refractory epilepsy. Interictal FDG PET-computed tomography done for lateralization and localization of seizure focus showed right-sided frontoethmoidal encephalocele associated with hypometabolism in the adjacent right frontopolar cortex, concordant with ictal onset on electroencephalogram.