Background <p>Recurrent epistaxis, which frequently results in iron deficiency anaemia and impaired quality of life, is the most frequent complication of hereditary haemorrhagic telangiectasia (HHT). Specific data to guide rare disease management during a pandemic such as COVID-19 are lacking.</p> Methods <p>To better define the impact of the COVID-19 pandemic on HHT, we conducted a retrospective and prospective observational descriptive review of HHT patients in central South Africa. Epistaxis severity scores (ESSs) and haemoglobin (Hb) levels before and after the start of the pandemic were compared. Variables that may have influenced epistaxis severity and anaemia were investigated, viz. (i) mask use, (ii) nasal versus oral swabs to test for SARS-CoV-2, (iii) COVID-19 disease and (iv) its management, (v) COVID-19 vaccines, and (vi) the social impact of the pandemic.</p> Results <p>Twenty-four patients with confirmed HHT were included in the study. Subset analyses revealed a clinically significant change in ESSs (≥ 0.71 minimal important difference) and Hb levels (&gt; 2.7% biologic variation) in 6/11 (54.6%) and 12/15 (80%) patients. While the median ESS improved in 2/11 (18.2%) patients, it worsened in 4/11 (36.4%) patients during the pandemic. However, the changes in the median ESS (2.25 pre-pandemic versus 2.5 during the pandemic; p = 0.38) and Hb level (9.5&#xa0;g/dL pre-pandemic versus 10&#xa0;g/dL during the pandemic; p = 0.38) for the study population were not statistically significant. Clinical and social variables that may influence epistaxis severity and anaemia were identified. Nasal swab testing for SARS-CoV-2 induced epistaxis in 9/12 (75%) cases and was noted as an important factor.</p> Conclusion <p>The COVID-19 pandemic has had a clinically significant impact on epistaxis severity and anaemia in some individuals with HHT in central South Africa. Specific strategies are needed to optimise the management of HHT during the COVID-19 and future respiratory pandemics.</p>