Takotsubo cardiomyopathy, a disease that causes transient contractile abnormalities mainly in the left ventricular apex, is rarely reported in children, especially in those with single-ventricle disease. A 4-year-old boy with a single right ventricle was transferred to our hospital following a severe seizure and was diagnosed with takotsubo cardiomyopathy by echocardiography. His cardiac function improved; however, he developed hypoxic-ischemic encephalopathy.