Simultaneous primary brain tumors in pediatric patients without prior chemotherapy or radiotherapy, phacomatosis, or known familial history are a rare occurrence. The authors report the case of a 4-year-old boy with simultaneous choroid plexus carcinoma and pilocytic astrocytoma with features of oligodendroglioma. Magnetic resonance imaging studies revealed diffuse heterogeneously enhancing left intraventricular and posterior fossa tumors initially believed most consistent with multicentric choroid plexus carcinomas. A multiple staged resection was carried out for each tumor and gross-total resection was achieved. Upon gross inspection intraoperatively as well as postoperative histological analysis, 2 distinct simultaneous tumors were identified: choroid plexus carcinoma and pilocytic astrocytoma. To the authors' knowledge this is the first case report published identifying 2 distinct tumor types with similar radiological appearances in a pediatric patient with no prior history of radiotherapy, chemotherapy, or phacomatosis.