The case of an 18-year-old man with a tumor of the left lateral ventricle is reported. The mass was observed to be contiguous with the choroid plexus but could be totally removed. Pathological examination disclosed an inflammatory pseudotumor. The authors review the literature on this lesion and related disorders involving the central nervous system and stress the need for a morphological differential diagnosis. Inflammatory pseudotumor should be considered in any case of intraventricular tumor.