The authors report a case of a female baby born with large congenital scalp and skull defects with exposure of the cranial arachnoid mater. The female patient was delivered by Caesarean section following diagnosis of a large cranial skin defect by ultrasound. The skin defect was present at the time of birth, measured 8.5 cm × 8.0 cm, and involved the full thickness of the cranium. The cranial arachnoid mater was exposed and veins running on the surface of the brain were observed. There was no means to cover the large defect surgically and conservative treatment was initiated. Complete epithelialization of the scalp defect was acquired without fatal problems. In the course of treatment, Mepilex Transfer was very effective. The repair of congenital defects of the scalp and skull is a major challenge not only because of the rarity but also because of severe morbidity. A surgical or conservative approach is chosen according to the situation. This rare case highlights two important clinical issues: that the congenital defects of the scalp and skull could not be managed with a surgical approach and in its place, a conservative approach using the special dressing Mepilex Transfer proved highly effective.