Spinal epidural haematoma (SEH) is a rare complication in haemophiliacs. We report the case of a 17-year-old boy with severe haemophilia B who presented with acute onset of neck/back pain, walking impairment and urinary retention because of an extensive SEH. The haematoma was identified by magnetic resonance imaging of the spinal column. Prompt and aggressive treatment with factor IX concentrate led to complete recovery at 3 weeks. This case calls attention to the clinical manifestation, radiological features and management options of the rarely reported SEH in haemophiliacs. Despite evidence of extensive SEH, factor replacement therapy and a multidisciplinary team approach result in complete neurological recovery without the need for surgical decompression.