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Choroid plexus papillomas (CPPs) are rare neoplasms classified as World Health Organization grade I tumors. CPPs containing other tissues have occasionally been documented in the literature. However, few of these previous reports have provided clinical and radiological information. We herein report a case of a posterior fossa CPP with focal ependymal differentiation in a 42-year-old woman who presented with a 6-month history of progressive headache. Preoperative radiological images showed a hypervascular tumor protruding into the left foramen of Luschka with perilesional edema. Gross total resection of the tumor was performed. Histopathological examination revealed that the tumor was composed of papillary structures. Immunohistochemical staining of glial fibrillary acidic protein was focally positive around the capillaries, which was suggestive of “perivascular pseudorosette” formation. Our case showed similar imaging appearances as those of CPP; thus, it seems difficult to distinguish CPP with versus without ependymal differentiation by clinical and radiological features alone. The clinical significance and pathogenesis of ependymal differentiation in CPP remain unclear, and further case reports are required. Keywords: Choroid plexus papilloma, Ependymal differentiation, Fourth ventricle, Magnetic resonance imaging