Patients with normal-flow low-gradient (NFLG) severe aortic stenosis present both diagnostic and management challenges, with debate about the whether this represents true severe stenosis and the need for valve replacement. Studies exploring the natural history without intervention have shown similar outcomes of patients with NFLG severe aortic stenosis to those with moderate aortic stenosis and better outcomes after valve replacement than those with low-flow low-gradient severe aortic stenosis. Most studies (all observational) have shown that aortic valve replacement was associated with a survival benefit vs surveillance. Based on available data, the European Society of Cardiology/European Association for Cardio-Thoracic Surgery guidelines and European Association of Cardiovascular Imaging/American Society of Echocardiography suggest that these patients are more likely to have moderate aortic stenosis. This clinical entity is not mentioned in the American Heart Association/American College of Cardiology guidelines. Here we review the definition of NFLG severe aortic stenosis, potential diagnostic algorithms and points of error, the data supporting different management strategies, and the differing guidelines and outline the unanswered questions in the diagnosis and management of these challenging patients.