Isolated levocardia (also known as situs inversus with levocardia) is a rare type of organs' situs inversus in which the heart is still in normal position but other abdominal viscera are transposed.[1][2] Isolated levocardia may occur with heart defects and patients without having operations have low life expectancy: only about 5% to 13% of patients survive more than 5 years.[3] Therefore, even though the risk of cardiac surgeries is high, once patients are diagnosed, operations are suggested to be held as soon as possible. Isolated levocardia is congenital. So far, there is not sufficient evidence to prove that chromosome abnormalities will result in isolated levocardia, and the cause of isolated levocardia is still unknown.