A 65-year-old man was referred for evaluation of a cardiac murmur. The patient reported decreased effort capacity and dyspnea. Cardiac examination revealed a grade 3–4 diastolic murmur at the aortic area and a blood pressure of 145/45 mmHg. A comprehensive metabolic panel and lipid profile were within normal limits. The patient did not have a history of smoking. He was tall of stature and had enlarged hands and facial bones, which suggested acromegaly. These features had been noted for at least 10–15 years by the patient’s family members. An echocardiographic examination revealed an enlarged ascending aorta (4.8 cm) with moderate to severe aortic regurgitation and a hypertrophied and enlarged left ventricle (end diastolic diameter 72 mm) with preserved systolic function. The aortic valve was apparently normal and the cause of aortic regurgitation was the ascending aortic aneurysm with dilated aortic annulus, namely, annuloaortic ectasia. A computed tomographic scan confirmed the ascending aortic aneurysm and anuloaortic ectasia, with a maximum aortic diameter of 6 cm in the root and 5.6 cm at the sinotubular junction (Figs. 1, 2). Fasting growth hormone (GH) and insulin-like growth factor-1 (IGF-1) levels were increased to >40 ng/ml (normal range 0–1 ng/ml) and 930 ng/ml (normal range for age 75–212 ng/ml), respectively. GH did not decrease with glucose loading. Fasting blood glucose was within normal limits, but glucose loading revealed impaired glucose tolerance. A magnetic resonance image of the hypophysis showed a macroadenoma (18 × 17 mm2) of the anterior hypophysis (Fig. 3) consistent with a GH-secreting tumor. Coronary angiogram revealed normal coronary arteries. The patient was started on octreotide treatment and a composite graft of the ascending aorta and aortic valve replacement were performed.