Pineal region tumors are uncommon lesions in the central nervous system. Papillary tumor of the pineal region is recently recognized as a separate disease. Its incidence, treatment, and outcome are not well-defined. We have reported the case of a 6 yearold- boy with papillary tumor of the pineal region. He presented with headaches, nausea and vomiting and, after a biopsy, was referred for radiotherapy. The patient received 54 Gy irradiation to the pineal region followed by a chemotherapy regimen of cisplatin, vincristine, and lomostine. His tumor decreased slightly. After 4 years, the patient has remained well and attends school. He is under routine follow-up. We suggest radical radiotherapy as the main treatment for papillary tumors of the pineal region.