We present here an unusual case of papillary neuroepithelial tumor of the pineal region. The patient was a 29-year-old female who presented with headaches. A computed tomography scan revealed a tumorous lesion at the pineal region and hydrocephalus. The resected tumor was composed of columnar and cuboidal cells showing characteristics of papillary growth. The tumor cells exhibited diffuse and intense immunoreactivity to cytokeratins and neural cell adhesion molecule. The tumor expressed abundant levels of transthyretin (prealbumin) and appeared ependymal in nature, with numerous microlumens delineated by punctate and ring-like patterns in epithelial membrane antigen staining. Reactivity to synaptophysin and glial fibrillary acidic protein was observed only in the infiltrated non-neoplastic pineal parenchyma. These histological characteristics matched the description of the recently reported papillary tumor of the pineal region thought to originate from the specialized ependyma of the subcommissural organ (SCO). Transthyretin expression of the present case further supports the likelihood of SCO origin, as transthyretin is one of the proteins presumed to be secreted by human SCO.