The atypical choroid plexus papilloma(CPP)is a new category of choroid plexus tumor recently added in the 2007 WHO classification of tumors of the central nervous system. We report the case of a 64-year-old female patient with atypical CPP of fourth ventricle. The patient presented with a 2-month history of dizziness and gait disturbance. Imaging studies showed a large tumor in fourth ventricle. We did suboccipital craniotomy with tumor removal. Pathologic study revealed atypical CPP. Based on our knowledge, up to now, this patient is the first atypical CPP case of fourth ventricle in Korea.