A full-term newborn boy presented with a large full-thickness calvarial skin and skull defect in the vertex over the superior sagittal sinus (SSS), posterior to the coronal suture. No other malformation was found. The superior wall of SSS was only covered with a thin membrane. The superior wall of SSS was successfully reconstructed with bovine pericardium dura substitute (Lyoplant) and the calvarial defect was closed by a scalp rotational flap. Postoperative period went uneventful, and follow-up 1 year after surgery showed complete closure of the skin defect and a normal neurological development. The patient was protected from trauma with a tailored helmet. This is a rare case, which was successfully reconstructed with bovine pericardium dura substitute with no complications related to the reconstruction material.