In female patients affected by congenital coagulation disorders haemorrhagic corpus luteum must be considered for the differential diagnosis of acute abdomen. We report the complication in a 22-year-old woman with type 3 von Willebrand's disease who presented with abdominal pain and haemorrhagic shock. Along with adequate factor VIII replacement therapy oral contraceptive treatment is the key for the management of this potentially life-threatening complication and the long-term prevention of future bleeding episodes of this origin.