Abstract Myelomeningocele may be associated with other neural and extraneural anomalies. Authors present association of metopic suture abnormality, an interfrontal encephalocele with widening of metopic suture and abnormal shape frontal bones in the forehead in those associated with hydrocephalus. Authors describes two neonates with interfrontal encephalocele, representing first series reporting in neonate. Management and pertinent literature is briefly discussed.