A 70-year-old man with sudden onset of dyspnea was referred to our emergency room. Over several years, he had received medical treatments for hypertension, diabetes mellitus, hypercholesterolemia, and obesity, and had a history of heavy smoking until the age of 60. In the past month, systolic blood pressure (BP) increased from around 120/- mmHg to 170/- mmHg or over and exertional dyspnea appeared. On admission, BP was 241/134 mmHg and pulse rate was 140 beats per minute. The chest X-ray showed severe pulmonary edema with butterfly shadow and blood gas analysis revealed the PO2 was 62.2 mmHg, the PCO2 57.7 mmHg, and the pH 7.208 with oxygen inhalation of 12 litters per minute. These were the typical manifestations of clinical scenario (CS) 1 of heart failure (HF). Chemical analysis showed total cholesterol of 240 mg/dl, LDL-cholesterol 159 mg/dl, triglyceride 127 mg/dl, creatinine 1.04 mg/dl, BNP 455.7 pg/ml, and plasma renin activity over 20 ng/ml/hr. Intravenous nitroglycerin, atrial natriuretic peptide and furosemide and noninvasive positive pressure ventilation with oxygen inhalation were consecutively administered. The symptom rapidly resolved thereafter. The left ventricular ejection fraction (EF) of 25–30 % on admission improved to 66 % 5 days later. Abdominal ultrasonography showed an atrophic left kidney (pole-to-pole length 77 mm, resistive index 0.61) compared with the right kidney (pole-to-pole length 96 mm, resistive index 0.71), which suggested the stenosis of the left renal artery and plain computed tomography showed severe calcification of the abdominal aorta and left renal artery. Contrast-enhanced magnetic resonance imaging and diagnostic angiography revealed severe left renal stenosis. There was no significant stenosis in any coronary arteries. After renal revascularization, BP decreased and antihypertensive agents were reduced, and there was no exacerbation of HF afterward. Renovascular hypertension (RH) is a rare disease in daily practice and is recognized as one of the causes of HF with unknown etiology. RH with recurrent exacerbation of HF is the indication for renal revascularization. However, renal artery stenosis is rarely identified even in HFpEF (HF with preserved EF) patients. We experienced a de novo HFpEF case with the typical features of CS 1 of hypertensive emergency complicated by RH with underlying multiple arteriosclerotic diseases. The association between de novo typical CS-1 heart failure of hypertensive emergency and renal artery stenosis might be closer and thus both conditions might more often coexist than expected, and once RH would be identified, the clinical course could be carefully observed thereafter.