Patients with left ventricular outflow tract (LVOT) obstruction comprise a diverse group of neonates, infants, children, and young adults. The patients may be critically ill or asymptomatic. In this article, the presentation, diagnosis, management, and outcomes are discussed in patients with coarctation of the aorta, hypoplastic left heart syndrome, valvar aortic stenosis, subaortic stenosis, and Shone's anomaly.