Summary

We report a case of 46-year-old Asian woman with a history of recurrent epistaxis who presented with dyspnoea on exertion. Physical examination revealed mucocutaneous telangiectasias and signs of heart failure. Further evaluation showed huge hepatic arteriovenous malformation and severe pulmonary hypertension. This case demonstrates an uncommon manifestation of hereditary haemorrhagic telangiectasia presented with severe pulmonary hypertension.