Xanthogranulomas of the choroid plexus are found relatively frequently at autopsy; however only 8 cases of symptomatic choroid plexus xanthogranuloma of the lateral ventricle have been reported. We describe an additional case in a 63-year-old man who presented with headache. A well circumscribed mass located in the trigone of the right lateral ventricle and extending into the periventricular white matter was detected on imaging studies. The clinical impression was metastasis. The resected material had the typical histological appearance of a xanthogranuloma of the choroid plexus. The clinical and histopathologic features of these lesions are reviewed and other xanthogranulomatous processes involving the brain are briefly discussed.