A series of 19 children with congenital nasal dermoid cyst or fistulas admitted in our department between 1978 and 1992 was reviewed in a retrospective study. They were 8 females and 11 males, ages ranged from 9 months to 7 years (mean: 2 years and 4 months). At diagnosis 8 children presented with infection (superficial in 6 cases, neurological in 2 cases), and in 11 cases the malformation was discovered by the family physician. In 14 cases the cyst was located at the mid nasal ridge. Four children had associated malformations: neurodevelopmental delay (2 cases), hypertelorism (1 case) and external ear agenesia with facial palsy. The cyst was subcutaneous in 5 cases and was operated on through a nasal approach. The 14 other children were operated through associated intra and extracranial approaches. Surgical exploration disclosed a dural extension of the fistula in 9 cases. When performed, preoperative radiological explorations including CT Scan and/or MRI had not always predicted the dural extension. The mean follow-up is 5.3 years. No recurrence was recorded and the cosmetic result was always excellent. Preoperative radiological investigations are necessary to precise the location of these malformations and to detect associated disorders, but they appear unable to prove the extension of the fistula to the dura. Intracranial and extracranial approaches should be always planned to perform a total excision of the lesion.