Hereditary Haemorrhagic Telangiectasia (HHT), also known as Osler-Weber-Rendu Syndrome, causes Arteriovenous Malformations (AVM), increasing the risk of haemorrhage. It is inherited in an autosomal dominant fashion and has high penetrance, estimated around 95% by late adulthood. Prevalence is approximately 1:10,000 in North America. The condition commonly presents with recurrent epistaxis in teenage years, followed by the presence of telangiectasia (small AVMs) on the lips, tongue and gastrointestinal tract mucosa in adulthood. Larger AVMs can occur in the brain, lungs and liver, occasionally causing life-threatening complications. Gastrointestinal involvement may lead to occult or overt bleeding per rectum and iron deficiency anaemia. The management of HHT largely depends on the site and symptoms of the AVMs. Although an awareness of the multi-organ pathology HHT can cause is important, epistaxis management is the challenge that ENT practitioners face. Simple measures such as regular humidification or moisturisers are followed by medical treatments, including oral antifibrinolytic or systemic antiangiogenic agents. Finally, there are a range of surgical approaches to control epistaxis, depending on the severity of symptoms.