This report concerns a 24-year-old male patient with Down syndrome, complicated by a benign but true neoplastic lesion of a differentiated papilloma of the choroid plexus which simultaneously displayed a dysgenetic character. A review of the literature revealed a lack of single case reports of Down syndrome with brain tumors, which may reflect chance occurrence; however, the brain tumors in Down syndrome patients are characterized by their dysontogenetic or dysgenetic character.