Frontoethmoidal meningoencephalocele is a rare congenital disease where intracranial masses protrudes through a midline defect at foramen coecum from the anterior cranial fossa into the facial skeleton. The patient presents at birth with a soft-tissue swelling at the nasal bridge. In the majority of cases, the cele is completely covered by skin. The face is elongated, the nasal bridge is broad, the upper part of the nose is long, and the patients have telecanthus. Frequently, the patients have a normal psychomotor development, but may have intracerebral malformations and a shunt dependent hydrocephalus. Spinal fluid leakage may result in meningitis. Anosmia is infrequent. The patients may have a wide range of ophthalmological problems. Newborns with meningoencephalocele should be examined by a neurosurgeon. The investigation and work-up commences with a multidisciplinary approach. We present two patients with meningoencephaloceles.