Mitral regurgitation is the second most frequent valvular heart disease. Echocardiography is the principal examination to determine severity, mechanism and hemodynamic consequences of mitral regurgitation and consequently contributes to the assessment and accurate timing of the best treatment strategy. To clarify clinical decision making in severe mitral regurgitation, this review will discuss the diagnostic work-up and treatment options according to the most recent guidelines. Mitral valve surgery, preferably repair, is indicated in symptomatic patients with severe, organic mitral regurgitation. Chronic, functional mitral regurgitation is often medically treated (including cardiac resynchronization therapy if indicated), however surgery (preferably annuloplasty) can be recommended. Percutaneous MitraClip-implantation may be considered as an alternative option in symptomatic patients with severe mitral regurgitation who are considered inoperable. At present, there is no consensus on the optimal care in asymptomatic patients with severe, organic mitral regurgitation and preserved left ventricular function. A prospective trial is highly needed to elucidate this best treatment strategy.