Congenital afibrinogenaemia, a rare disorder of coagulation, is characterised by the congenital absence of fibrinogen. Uncontrolled umbilical cord stump bleeding is usually the first manifestation of the disease. Although various spontaneous haemorrhages may occur during lifetime, spontaneous epidural haematoma is rare. We report a case of congenital afibrinogenaemia in a paediatric patient who admitted to the emergency department with a complaint of headache. Computerised tomography scan of the head revealed a right parietal acute epidural haematoma. The patient underwent surgical evacuation subsequent to fibrinogen replacement in the emergency room. We decided to present our case since congenital afibrinogenaemia is rare in the emergency care. This report underlines congenital afibrinogenaemia as a potential cause of spontaneous acute epidural haematoma in paediatric patients which may require emergent intervention before the development of irreversible neurological conditions.