A 24-year-old woman with severe haemorrhagic complication due to type III von Willebrand's disease is reported, as the first such case in Hungary. Analysis of seven family members affected by the type III mutant gene was compatible with an autosomal recessive mode of inheritance. Severe, iatrogenic epistaxis of the patient was not improved by cryoprecipitate and DDAVP. Platelet suspension together with cryoprecipitate immediately stopped bleeding. Simultaneous application of cellulare (platelet suspension) and plasmatic (cryoprecipitate) Willebrand protein is suggested for patients with severe (type III) von Willebrand's disease.