Xanthogranulomas of the choroid plexus in the lateral ventricle are usually asymptomatic lesions found incidentally at autopsy. Symptomatic cases are rare. We present the case of a 30-year-old woman with a xanthogranuloma of the right lateral ventricle who showed intracranial hypertension and visual disturbance. Plain T1-weighted magnetic resonance images disclosed a isointense right temporal lesion in the ventricular horn which showed decreased signal intensity on T2-weighted images and massive enhancement on T1-weighted images after gadolinium injection. She was operated on through a right temporal craniotomy with total excision. We review the literature and discuss radiological findings on MRI.