Background: Schizencephaly is a rare defect which is identified as clefts that are lined with grey matter extending from the ependyma of the cerebral ventricles to the pia mater. An encephalocele occurs due to failure of neural tube closure resulting in a gap through which cerebrospinal fluid and meninges can bulge into a pouch. There have been rare instances when these two defects have presented simultaneously. Case Description: We report a case of a 17‑year‑old child who was brought by his parents with complaint of swelling over his nose and forehead and aggressive behavior since birth. Magnetic resonance imaging findings were consistent with frontoethmoidal meningoencephalocele with schizencephaly. Lumbar drain was inserted and kept in place for 1 week followed by surgical correction of the defect. Our case is interesting because of delayed presentation as it is a rare entity and its association with schizencephaly. Conclusion: Encephalocele association with schizencephaly is rare.