Papillary tumor of the pineal region (PTPR) is a recently described distinct clinicopathological entity. The purpose of this case report is to increase the knowledge of its neuroradiological findings and natural history by describing the long-term clinical and neuroradiological follow-up of a PTPR occurring in a 56-year-old Italian male patient. At magnetic resonance imaging (MRI) obtained at diagnosis, the lesion showed a subtle high signal intensity on T1-weighted imaging. Twenty-nine months later, MRI showed clearcut enlargement of the lesion, which had only a small area of high signal intensity on T1-weighted images, and a minimum apparent diffusion coefficient of 0.854 x 10(-3) mm(2)/s. Treatment included surgery followed by irradiation. Three-month MRI follow-up did not show disease relapse.