Papillary tumor of the pineal region (PTPR) is a newly described entity, which has been recently included in the World Health Organization classification of central nervous system tumors. We report an unusual presentation of PTPR in a 17-year-old girl, which was extending into the third ventricle, along with a detailed description of morphological and immunohistochemical characteristics of PTPRs. The diagnosis of PTPR was established on immunohistopathological examination.