Two cases of dermoid cysts of the posterior fossa in association with a dermal fistula and with different clinical presentations are reported. The patient in case 1 is a 14-month-old girl with a history of recurrent bacterial meningitis. Case 2, a 7-year-old girl, presented with a skin "granuloma" at the inion. Both cases, and a review of the literature, demonstrate the need for a thorough exploration of the cranial and spinal midline skin areas in every newborn and argue for widespread use of nuclear magnetic resonance imaging whenever a suspected midline lesion is found.