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Benign chorioangioma of the placenta is the most common primary tumor of the placenta similar to hamartoma. Most small-sized tumors do not make any clinical problem, but uncommon large tumors (> 5 cm in diameter) may produce both maternal and fetal complications, such as polyhydramnios, preterm labor, fetal hydrops, microangiopathic hemolytic anemia, disseminated intravascular coagulation, intrauterine growth restriction, preeclampsia and placental abruption. In this respect, the diagnosis and management of chorioangioma and its complication should be done appropriately. We report a case of chorioangioma presenting with polyhydramnios, preterm labor, fetal anemia and fetal hydrops, diagnosed by antenatal ultrasonography and postnatal placental histologic examination, and live born baby with the brief review of the literature related to this type of tumor.