The congenital dermal sinus (CDS) is a benign tumor-like entity that has unique anatomical and clinical features. We retrospectively examined our data to determine factors associated with adverse outcomes for cranial-based dermal sinuses.We retrospectively examined our data obtained from patients presenting between 1975 and 2002. Sixty-seven patients with a CDS were found. Of these, 20 cases of a cranial CDS were identified, 15 of which had evidence of an intradural component.Surgical treatment of congenital dermal sinuses was accompanied with, in general, excellent results. Poor outcomes were most strongly associated with delays in diagnosis. The most common complications were infection at the surgical site (five patients) and hydrocephalus necessitating ventriculoperitoneal shunt placement (two patients). Permanent neurological deficits were rare.Cranial CDSs are unusual lesions found in children. A majority of these lesions may penetrate the dura and lead to significant complications if not promptly identified and treated in an appropriate and timely fashion.