Heart failure with a normal ejection fraction (HFNEF) is a common clinical problem with many unsolved questions regarding pathophysiology, diagnosis, and therapy. Although the term diastolic heart failure has been abandoned, diastolic left ventricular (LV) dysfunction together with combined systolic ventricular and arterial stiffening are considered to be the main pathophysiologic mechanisms in HFNEF. Current guidelines define HFNEF by symptoms or signs of heart failure in the presence of LV ejection fraction of more than 50%, but with additional evidence of LV diastolic dysfunction. Definite diagnosis of HFNEF requires exclusion of valvular heart disease, constrictive pericarditis, and several noncardiac diseases. Echocardiographic assessment of the tissue Doppler-derived filling index E/E', which is the ratio of the peak early mitral valve flow velocity to the peak early diastolic mitral annular velocity, should improve the noninvasive estimation of filling pressures in suspected HFNEF. Elevated plasma levels of natriuretic peptides may confirm HFNEF if tissue Doppler echocardiography is inconclusive. Treatment of HFNEF is symptom oriented and mainly based on pathophysiologic assumptions such as heart rate reduction, blood pressure control, and maintenance of sinus rhythm. In contrast to heart failure with reduced ejection fraction, large-scale randomized controlled drug trials for HFNEF are scarce and could not demonstrate mortality reduction so far.