BACKGROUND Chorioangiomas are the most common benign tumors of the placenta originating from primitive angioblastic tissues. It comprises near 1 percent of placental tumors. Clinical manifestations in the newborn are rare and usually associated with tumors greater than 5 cm in diameter and consist of polyhydramnios, fetal anemia, massive edema with pleural effusion, ascites and intrauterine growth retardation. We present a case with large chorioangioma as the cause of non-immunologic hydrops fetalis with a successful outcome. METHODS The patient was a female newborn with history of polyhydramnios, symptoms of congestive heart failure and associated anemia, thrombocytopenia and coagulopathy. The pathophysiology and management of the complications of hydrops fetalis with chorioangioma are discussed CONCLUSIONS Chorioangioma of the placenta has potentially serious perinatal risks and so the pregnancy needs to have regular surveillance. The chance of developing complications is directly related with the tumor size.