Except for villous hypertrophy of the choroid plexus that may not be true tumors, multiple choroid plexus papillomas are extremely rare. The authors report a case involving multiple choroid plexus papillomas that were distinct from villous hypertrophy. These lesions were localized, one in the atrium of the right lateral ventricle and the other in the inferior horn of the left lateral ventricle. A review of the literature revealed that this case represented the first reported case of true multiple choroid plexus papillomas documented by findings on magnetic resonance imaging.