Placental chorioangioma and thrombosis of an umbilical vein varix are rare etiologic factors of non-immune hydrops fetalis. Herein, we report a patient who had hydrops fetalis associated with placental chorioangioma and thrombosis of an umbilical vein varix. This is the first report of coexistence of non-immune hydrops fetalis with placental chorioangioma and thrombosis of an umbilical vein varix.