There are few data concerning long QT syndrome (LQTS) in fetuses, neonates, and infants. Although most patients with LQTS do not have significant symptoms at this age, those who present with severe symptoms in early life have a worse prognosis. Early recognition of these symptoms as manifestations of LQTS and institution of appropriate therapy may prevent sudden cardiac death and SIDS in these children. Unfortunately, traditional medical therapy may not be effective in the most at-risk children and more aggressive management is needed; however, because of the size of these children, innovative approaches to providing this therapy are needed.