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The pericardium was absent and the great vessels were exposed. There was an epigastric omphalocoele and a ventral diaphragmatic defect. The diagnosis of Pentallogy of Cantrell was made. The challenges involved in the evaluation and management of this case in a Nigerian setting are discussed.doi:10.4314/njp.v39i1.7 fatcat:7au5jp7ywzbrlkull7brhek3b4