Congenital intrapericardial teratoma is a rare, usually benign tumour frequently associated with massive pericardial effusion, cardiac compression, and severe cardiorespiratory distress shortly after birth. Surgical removal is not only curative but also potentially lifesaving because these lesions often become fatal if not promptly excised. We present a case of a newborn with a huge intrapericardial teratoma diagnosed in utero. After normal delivery the infant underwent surgical removal and has had clinical follow-up for more than 11 months.

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