Intracranial haemorrhage (ICH) is the most serious event in haemophiliacs, resulting in high rates of mortality and disability. Although the use of a prophylaxis regimen has improved outcomes, the mortality caused by ICH is still around 20%. ICH is more frequent at two different ages: in childhood (mostly in children aged ≤2 years) and in adulthood (with known risk factors such as hypertension and age ≥60 years). Our review shows how ICH remains one of the worst problems of patients with haemophilia. Greater attention to risk factors and early symptoms, together with an appropriate early prophylaxis, may reduce the risk of severe intracranial haemorrhagic events.