Background Pericardial defects are rare anatomical variations that can present as an isolated variation or be associated with other conditions. They are usually asymptomatic and misdiagnosed conditions, and given their rarity, partial pericardial defects can have devastating outcomes. The sudden death of an apparently healthy newborn certainly raises concerns, and a medico-legal investigation is crucial in establishing the cause of death. This case report highlights the importance of awareness on the part of obstetric professionals of the lethal outcomes of pericardial partial congenital defects. This case also demonstrates the difficulty of establishing a correct diagnosis. Case presentation The autopsy of a 15-h-old neonate revealed a partial pericardial defect ending in a biventricular strangulation by the defective pericardium. Other findings, such as the patency of the arterial ductus, a subarachnoid hemorrhage, and aspiration of amniotic fluid, were also reported. Conclusions Although imaging techniques have evolved, fetal detection of cardiac abnormalities can be tricky, especially when occurring as an isolated variation.
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