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A rare mirror syndrome (Ballantyne syndrome) was seen in a woman who carried a hydropic fetus caused by fetal atrio-ventricular septal defect (AVSD). Diagnosis was made with confidence after ruling out cardiogenic pulmonary edema and preeclampsia. Placental pathology demonstrated multifocal villous edema and accelerated maturation of trophoblasts which may support the earlier reports about potential etiologic roles of the placenta to trigger the disease.