Hereditary hemorrhagic telangiectasia (HHT) also known as Rendu Osler syndrome is a vascular hereditary autosomal dominant disease, leading to a dysfunction in the development of arteriovenous capillaries, usually resulting in epistaxis, gastrointestinal (GI) bleeding, and iron deficiency anemia. It is believed that by interfering and stopping angiogenesis, anti-vascular endothelial growth factor molecules could be an option for HHT patients. Indeed, an intranasal treatment regime of diluted Avastin (bevacizumab; recombinant humanized antivascular epithelial growth factor immunoglobin G1) has proven clinically efficacious in patients with HHT1. However, there are no data available regarding bevacizumab's effect in patients with HHT and GI bleeding. We report here the case of an 85-year-old woman, suffering from life-threatening GI bleeding due to HHT with an impressive clinical response using anti-vascular endothelial growth factor infusion.