A unique case of a neonate with agenesis of the fibrous pericardium combined with Bochdalek's foramen presenting herniation of a solitary cyst of the liver into the left hemithorax is presented. It is emphasised that the lack of the pericardium has not interfered with normal function of the heart, one year after the operation. The possible pathogenesis and embryology of this combination of malformations is discussed.
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