W e present a 70-year--old man who had a past medical history of dilated idiopathic pulmonary artery, incidentally diagnosed at the age of 23 years. He was asymptomatic until a year ago when he was admitted for an episode of anginal chest pain in the context of paroxysmal atrial fibrillation. At rapid rates, he showed pathological ST segment descent. A coronary angiography showed a slight stenosis of the left main coronary artery (LMCA). At that time, the mean pulmonary artery pressure was 36 mm Hg. After recovering sinus rhythm, the patient became asymptomatic and was discharged with β-blockers.A year later, the patient was admitted with resting angina. The echocardiogram ( Figure A) and the computed tomography-angio showed that the aneurysm had increased to 80 mm and compressed the LMCA (Figure B). This finding was confirmed on coronary angiography and intravascular ultrasound that showed a critical stenosis of LMCA ( Figure C, insert 1, and online-only Data Supplement Movie I) and normal luminal diameter of anterior descending artery ( Figure C, insert 2). In addition, the mean pulmonary artery pressure had increased >60 mm Hg.Because of the high surgical risk posed aneurysm surgery, percutaneous revascularization of LMCA with drug-eluting stent was performed with good final result ( Figure D, insert 1, and online-only Data Supplement Movie II). We also initiated specific treatment of pulmonary hypertension (PH) with phosphodiesterase-5 inhibitors and an endothelin receptor antagonist. The patient became asymptomatic and was discharged to reevaluate the surgical indication of aneurysm according to the clinical and PH evolution in the follow-up. One year later, the patient is asymptomatic with no adverse events.Extrinsic compressions of LMCA by a pulmonary artery aneurysm are extremely rare and usually associated with PH. Only case reports and small series have described this entity as a cause of angina and sudden death, and, currently, the appropriate management of these patients remains unknown.