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Pentalogy of Cantrell is a rare a congenital syndrome which includes ectopia cordis, sternal, pericardial, abdominal wall defects (usually omphalocele), diaphragmatic defects and intracardiac abnormalities. We report a case with a rare combination of ectopia cordis omphalocele, dysplastic kidneys, megabladder and a malrotated pelvis in a 23‑years old woman who had come for an antenatal checkup in her 25th week of gestation.