Neurofibroma of the scalp are mostly multiple as part of neurofibromatosis or other phakomatosis. De novo solitary types are less common and rarely erode the skull, unlike the intracranial counterpart. Skull erosion has been reported in adults with longstanding plexiform neurofibromas. We report a giant neurofibroma on the scalp of a five-year-old boy, managed in our center. Although this condition is a rare entity, it should be anticipated and the treatment strategy should include repair of the skull defect.