Haemophilia A and B are the most common of the inherited bleeding disorders. Haemophilia in the newborn presents a number of challenges in terms of both diagnosis and management which reflect features unique to this age group. In the presence of a family history of haemophilia optimal management requires close co-operation between three individual specialist groups - obstetricians, haematologists and neonatologists, who each have an important role to play in ensuring a safe outcome for these infants. More problematic is where a family history is absent or has not been adequately elucidated in which case the diagnosis of haemophilia in the neonate will be unsuspected. Diagnostic difficulties may then arise due to failure to recognise the presence of abnormal bleeding, which is often different from that typically observed in older children with haemophilia. In addition, diagnostic investigations are complicated by physiological differences in the neonatal haemostatic system. Although major bleeding is relatively uncommon, the incidence of intracranial haemorrhage is higher during the first few days of life than at any other stage in childhood, which relates to the trauma of delivery. Controversy, however, remains on optimal strategies for both prevention and management of this potentially devastating complication.