SUMMARY
Nasal glial heterotopia (NGH) is a rare congenital, non-neoplastic displacement of cerebral tissue in extracranial sites. Together with a case report of NGH, we present the first systematic review of all published cases in order to summarise the relevant clinical findings and appropriate therapy, making the available evidence accessible to decision makers. A total of 72 original publications including 152 NGH cases were identified. The male:female ratio was 3:2. Most patients were children under 18 years (130 patients) and only 8% of cases were diagnosed in adults. The main clinical presentation forms were asymptomatic masses around the nasal root as well as nasal congestion. Magnetic resonance imaging was performed in 39% of patients, computed tomography in 22% of patients and a combination of both in 20% of patients. A diagnostic biopsy was performed in only 7 patients. All patients underwent surgical treatment and recurrence was reported in 14 patients within the first year of follow-up. In conclusion, NGH should be considered as a differential diagnosis of nasal masses in children. MRI is mandatory in order to exclude a connection to the central nervous system. Complete resection is curative treatment.