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On a rare occasion, pulmonary artery dilatation can be complicated by an extrinsic compression of the left main coronary artery (LMCA) whose effects are immediately evident, whereas a delayed presentation is unusual. We report the uncommon case of a delayed acute coronary syndrome caused by the extrinsic compression of the LMCA due to pulmonary artery enlargement and the potential problems related to its management. An 82-year-old woman with a history of severe chronic obstructive pulmonary disease, a previous episode of deep venous thrombosis and a computed tomography-documented pulmonary artery dilatation was referred to the emergency room for worsening dyspnoea and chest pain. Five days after admission to the coronary care unit, the patient developed a cardiogenic shock with consecutive episodes of ventricular fibrillation. Urgent coronary angiography showed severe LMCA stenosis caused by extrinsic compression from the pulmonary artery with no other lesions in the coronary arteries; coronary angioplasty was successfully performed with a direct drug-eluting stent implantation that led to a significant improvement of the haemodynamic conditions in the following days. Planned control angiography performed 10 days later showed the recurrence of the LMCA stenosis together with a forward displacement of the previously implanted drug-eluting stent, which was managed with a further direct implantation of a bare metal stent. The immediate good results of this second procedure were confirmed by follow-up angiography performed 2 months later and by the 6-month follow-up clinical examination.