Early identification of congenital heart diseases, specifically those affecting the structural integrity and function of the interventricular septum, in childhood is important toward decreasing the morbidity and mortality of those affected. We review the pertinent clinical and imaging manifestations for those with ventricular septal defects, ventricular septal aneurysms, tetralogy of Fallot, and hypertrophic (obstructive) cardiomyopathy, in addition to discussing first-line imaging studies, including echocardiography, and indications for advanced imaging.