Papillary tumor of the pineal region (PTPR) is a rare brain tumor that probably arises from ependymal cells. There are no known systemic treatments for PTPR once it is refractory to surgery and radiation. We present the first case of a durable radiographic and clinical response of a PTPR to bevacizumab, an antibody against vascular endothelial growth factor, despite multiple prior treatments. Bevacizumab may be an effective treatment for PTPR.