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Abstract Proximal interruption of the pulmonary artery (PA) is a rare congenital vascular anomaly with varying presentation. These patients can be asymptomatic or symptomatic with breathlessness, haemoptysis, recurrent chest infections and pulmonary hypertension. Here, we present a patient who presented with massive haemoptysis secondary to interruption of the left lower lobe PA. To the best of our knowledge, massive haemoptysis due to isolated interruption of the left lower lobe PA has been rarely reported in the English medical literature.