Osler-Weber-Rendu syndrome (OWRD) is a rare vascular dysplasia of genetic origin and of autosomal dominant transmission, the pathophysiology of which involves the vascular endothelial growth factor (VEGF), a major growth factor of angiogenesis. Bevacizumab is an anti-VEGF monoclonal antibody that has demonstrated its interest in this indication. Two patients, with a personal and familial history of haemorrhagic syndrome, who had an OWRD detected in recurrent epistaxis, cutaneous-mucosal telangiectasia, and complicated visceral arteriovenous malformations with an iron deficiency anemia. Due to the persistence of the symptomatology despite a transfusion support, the treatment with bevacizumab was started and allowed a favorable outcome. Bevacizumab appears to be very well tolerated and represents a therapeutic advance in OWRD.