Subaortic stenosis carries considerable morbidity and mortality. In most cases, patients have an underlying left ventricular outflow tract morphology that promotes turbulence at the outflow tract, which induces the development of subaortic fibromuscular tissue. A subset of patients will progress to develop severe stenosis and aortic regurgitation, but it has been difficult to determine which patients are at risk. While resection of the subaortic tissue improves immediate outcome, many patients have recurrence of both stenosis and regurgitation, questioning the efficacy of surgical intervention in asymptomatic patients. This review article describes the current understanding of the etiology, treatment, and prognosis of subaortic stenosis.