Papillomas of the choroid plexus are rare tumors of neuroectodermal origin; they represent less than 5% of all central nervous system (CNS) tumors in pediatric patients. Choroid plexus carcinomas are even rarer. We reviewed the incidence of these neoplasms at our reference hospital and found six tumors of the choroid plexus (five papillomas and one carcinoma) in five patients. Patient age ranged from prenatal to 25 months. All five patients underwent computed tomography (CT) examination. Four perinatal patients underwent ultrasound examination, four magnetic resonance imaging (MRI), and one (years ago) angiography. All patients had tumors located in the lateral ventricles, and one patient had a second tumor located in the third ventricle. These tumors are predominantly solid, intraventricular, with well-defined polylobulated margins. They show intense vascularization on Doppler studies and marked contrast enhancement on CT and MRI studies. Hydrocephalus was present in three cases. All patients underwent surgery; total resection was achieved in the five papillomas, whereas the carcinoma was partially resected and the patient is currently undergoing chemotherapy. The three patients with a single papilloma are disease free at follow-up (range 7 months to 11 years). The patient with two papillomas shows good recovery at follow-up, whereas the patient with carcinoma of the choroid plexus has a poor prognosis.