Intracranial hemorrhage of term neonates with severe von Willebrand disease type 3 and near normal delivery have not been published to date. We report on a term newborn with severe type 3 VWD who suffered a large intracranial bleeding presumably subsequent to sinus venous thrombosis. This case is remarkable in two aspects: i) with respect to the low risk of perinatal intracranial hemorrhage even in neonates with VWD type 3 and ii) since VWD type 3 with factor VIII:C levels < 5 IU/dl does not seem to protect from thromboembolism. Guidelines for the management of hemophilia advise atraumatic delivery of neonates with known or suspected bleeding disorders, however primary caesarean section in the absence of complications is not obligatory. To answer the question whether the presented case could have an impact on the recommended management we carried out a worldwide survey including 24 participating specialized centers. Except a single case from Sweden of a female neonate with VWD type 3 who also had an inhibitor against VWF no further cases were reported to us. We conclude that CNS bleeding in term neonates with severe VWD type 3 is a rare event that requires additional risk factors for manifestation. Therefore, the amendment of current guidelines does not seem to be mandatory.