The authors report a case of inflammatory pseudotumor that developed in the choroid plexus of the lateral ventricle. The patient was a 73-year-old man who had undergone surgery for rectal cancer at another hospital 5 years earlier. He was referred to the authors' department, with his chief symptoms consisting of disorientation, right hemiparesis, and gait disorder that had gradually developed during the preceding month. On computed tomography and magnetic resonance imaging, a well-demarcated and homogeneously contrasted tumorous lesion was noted in the region from the trigone to the medial wall of the inferior horn of the left lateral ventricle. Expansion of the inferior horn was also evident. Intraoperative findings showed that the tumor originated from the choroid plexus of the lateral ventricle, and the histopathological diagnosis was inflammatory pseudotumor. There are only 4 previously reported cases of inflammatory pseudotumor that developed in the choroid plexus; the authors review the literature and discuss the clinicopathological characteristics of the condition.