Background: In a monochorionic twin gestation, hydrops in one fetus often results from placental transfusion, accompanied by intrauterine growth restriction (IUGR) of the non-hydropic fetus. Case: A 35-year-old G11P9 presented with hydrops and IUGR of one of a monochorionic-diamniotic gestation. Extensive testing failed to reveal etiology for hydrops. The patient was delivered at 30 weeks gestation after non-reassuring fetal monitoring. The hydropic baby could not be adequately ventilated and expired in the early post-partum period. A diagnosis of superior vena cava syndrome and pulmonary hypoplasia secondary to right-sided congenital diaphragmatic hernia (CDH) was made neonatally. Conclusion: Right-sided CDH can be difficult to diagnose by sonography at times because of the echogenic similarity of liver and lung, and may be suspected from signs of pericardial effusion and ascites.

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