Occipital dermal sinuses (ODSs) are congenital lesions located in the midline and characterized by a cutaneous pit or dimple. The intracranial extension as well as the associated symptoms are variable. To date, a familial occurrence of these lesions has not been reported. In this paper the authors report on a 2-year-old boy with an ODS and intracranial hypertension. The boy's mother had a similar lesion but did not have any complaints. Following their experience with this case and a literature review, the authors suggest that there may be a genetic basis in certain instances of ODS.