Cardiac rhabdomyomas are typically presented in the tuberous sclerosis. Although benign and often associated with spontaneous regression, in rare circumstances huge mass size and critical location can lead to heart failure, ventricular outflow tract obstruction and refractory tachyarrhythmias. An 1-day-old girl was diagnosed as cardiac tumor during perinatal period. At birth, transthoracic echocardiography revealed huge cardiac mass located in septal area of both ventricle measuring 34×30 mm. It protruded into the left ventricular (LV) outflow tract, potentially obstructing it. A surface ECG revealed atrial tachycardia with nonsustained ventricular tachycardia with heart rate of 217 beats per min. The tachyarrhythmias were controlled with intravenous amiodarone. Reduction of the giant cardiac mass was treated with mammalian target of rapamycin pathway inhibitor sirolimus. However, she unfortunately died at 10 days-old because of sudden cardiac arrest maybe due to LV outflow tract obstruction during therapy. Gene analysis revealed TSC2 mutation after death. (Ewha Med J 2022;45(3):e5)

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