A term male infant was delivered by normal vaginal delivery following an uneventful pregnancy. He presented at 3 days of age with jaundice, weight loss, and poor feeding. He rapidly deteriorated with hypotonia, seizures, a tense fontanelle, and depressed conscious level. A cranial ultrasound scan showed moderate lateral and third ventricular dilatation, with no visible fourth ventricle. Doppler measurements showed absent diastolic flow, confirming markedly raised intracranial pressure. Cerebrospinal fluid pressure was markedly elevated and coagulation profile showed a specific prolongation of the activated partial thromboplastin time. Factor VIII assay confirmed haemophilia and a computed tomography scan showed a posterior fossa haemorrhage that was effacing the fourth ventricle and causing an acute hydrocephalus. He was managed with cerebrospinal fluid drainage and recombinant factor VIII concentrate. Posterior fossa haemorrhage is an unusual cause of acute high pressure hydrocephalus in the term neonate. Haemophilia should be considered as a cause of unusual intracranial haemorrhage in term neonates.