Acute myocarditis and ventricular arrhythmia are rarely seen as the initial presentation of systemic lupus erythematosus (SLE) in children. We reported the case of a 12-year-old girl with congestive heart failure, acute myocarditis and pericardial effusion as a primary manifestation of SLE. Sudden cardiovascular collapse due to ventricular fibrillation (VF), ventricular tachycardia (VT) and cardiac tamponade occurred. After resuscitation and pericardiocentesis, frequent VF/VT refractory to anti-arrhythmic therapy was supported by venoarterial extracorporeal membrane oxygenation. Early diagnosis and a combination treatment for heart failure, arrhythmias and immunosuppression may result in a favorable outcome.