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Choroid plexus carcinoma is a rare malignant brain tumor that occurs predominantly in childhood. A 203-day-old infant was admitted to our hospital with macrocephaly and right hemiparesis. The skull protruded diffusely in the left parietal area. Brain magnetic resonance imaging) revealed a huge mass in the left ventricle. We performed an open biopsy and discovered a choroid plexus carcinoma. The tumor bled very easily and hemostasis was difficult. After three cycles of chemotherapy, we resected the entire mass in a staged operation. Chemotherapy enabled us to resect the entire tumor by reducing its size. We report this case to stress the benefits of preoperative chemo-therapy and review the relevant literature. Key words:Choroid plexus carcinoma;Perioperative chemotherapy;Brain tumor.