Bilateral ligation of external carotids and ethmoidal arteries proved successful in controlling severe recurring epistaxis in a 13-year-old patient with Bernard-Soulier syndrome. In this child, epistaxis was the major bleeding symptom. Despite massive substitutive therapy, the patient suffered several life-threatening episodes of hypovolaemic shock. Although epistaxis is not reported as a cause of death in patients with haemostasis defect, our case might suggest that in selected cases with inherited platelet defect and intractable epistaxis so severe as to make normal or near-normal life impossible, surgical treatment could be considered.