The management of haemorrhagic episodes in patients with factor VIII inhibitor is difficult and the outcome rather unpredictable. The use of an investigational drug, that is, activated recombinant factor VII (rFVIIa) in a young non-haemophiliac patient with spontaneous occurrence of factor VIII inhibitor who presented with life-threatening retroperitoneal haemorrhage is reported. There was prompt achievement of haemostasis with rFVIIa after the patient had failed conventional therapy with factor IX and Autoplex. Two further episodes of retroperitoneal bleeding again responded promptly to rFVIIa therapy.