Intracardiac teratomas are rare primary tumours. We report the case of an infant prenatally diagnosed with an isolated multi-cystic mass developed in the right ventricle causing neonatal refractory ventricular arrhythmia. Despite rescue extracorporeal support and partial surgical resection, he died as almost all the previous reported perinatal intracardiac teratomas whatever the prenatal tolerance and the size of the tumour. The common poor outcome of fetal intracardiac teratomas should be known when counselling parents during pregnancy.
