Intraventricular tumors present a diagnostic challenge to the clinician because of a broad differential diagnosis with significant variability in tumor type between adult and pediatric populations. This expansive differential diagnosis includes choroid plexus papillomas and carcinomas, ependymomas, subependymomas, subependymal giant cell astrocytomas, central neurocytomas, meningiomas, and metastases as well as a number of cysts, inflammatory lesions, and other rare neoplasms. Posterior fossa ependymomas, subependymal giant cell astrocytomas, and choroid plexus tumors are more likely to appear in childhood, whereas subependymomas, central neurocytomas, intraventricular meningiomas, and metastases are more frequent in adults. This article reviews the epidemiology, the pathologic characteristics, and the primary diagnostic considerations of each tumor type.