Rendu-Osler-Weber disease is an autosomally dominant inherited disease which affects the connective tissue of blood vessels. The major symptom is recurrent epistaxis for which still exists no generally recommended treatment. Treatment modalities discussed in the literature include bilateral closure of the nostrils, brachytherapy, vessel embolization, hormone therapy, dermoplasties, as well as different kinds of laser therapies. Advantages and disadvantages of single treatment modalities are discussed. Dermoplasty and laser therapy seem to be the most suitable treatment procedures currently available for recurrent epistaxis in affected patients. Treatment carried out with the argon, KTP or ND:YAG laser has proven to be suitable as initial therapy. In most cases the additional use of long-term applications of a soft nasal ointment allows a lasting successful treatment. Our experiences show that dermoplasty should be reserved for cases with continued bleeding despite laser treatment but only rarely offers a long-term cure in the sense of a total recovery from epistaxis.