Objectives This systematic review aims to explore and outline the anaesthetic considerations and precautions for patients with a diagnosis of hereditary haemorrhagic telangiectasia (HHT) undergoing nasal surgery. Design A search was performed across four electronic databases between the dates 1 st January 2000 and 1 st March 2024. Two independent reviewers selected articles that met the following criteria: adult patients with HHT undergoing nasal surgery; description of anaesthetic regime; English language. Case reports and reviews were excluded. The Preferred Reporting Items for Systematic Reviews and Meta-Analysis guidelines were followed throughout. Results and participants Nine studies were included in the analysis, comprising a total of 800 patients. Two studies described local anaesthetic (LA) only, two described general anaesthetic (GA) only, and five described cases of each. Modality of anaesthesia was dependent on type of procedure (laser therapy, endoscopic surgery, sclerotherapy, or plasma coagulation) and patient factors (including HHT disease severity, vascular anatomy, and age). Anaesthetic approaches varied, although there were similarities; for example, six studies used a topical LA, combined with a vasoconstrictor. Only one study described a GA regime. Reduction of intra-operative bleeding was highlighted across multiple studies; measures included endotracheal tube/nasal lubrication, decongestants, and controlled hypotension. Complications were generally low, with three studies, totalling 218 patients, describing procedures in-clinic with LA with no reported complications. Conclusion The literature lacks clear consensus for selecting between LA or GA, as well as evidenced regimes for both. Therefore, further research is needed to better inform the anaesthetic approach for HHT patients undergoing nasal surgery.