Intracranial arteriovenous fistulas are vascular malformations in which clinical suspicion and prompt diagnosis, with a subsequent appropriate therapeutic approach, are crucial to avoid the development of irreversible neurological damage or even patient death because these lesions can be associated with heavy bleeding and high mortality rates. The authors present the case of a direct pial arteriovenous fistula in an infant and its unusual presentation as a large occipital protuberance with hard consistency and an audible murmur that produced bone remodeling. The patient was successfully treated by endovascular therapy, with complete regression of the occipital protuberance during follow-up.