Paediatrics, UCL Institute of Child Health, London WC1N 1EH.
Ehlers-Danlos syndrome predisposes to the development of vascular malformations, which may present as life-threatening haemorrhage. This article presents a patient with Ehlers-Danlos syndrome in whom spontaneous rupture of an unusual arteriovenous fistula occurred. This rare diagnosis can be reached through close attention to the history and examination.