We present the case of a large maxillary hemangioma treated by radiotherapy. Only 4 cases managed by radiotherapy have been reported in the literature, and most authors negate the radiosensitivity of the tumor. In our patient, the outcome was excellent; by 6 months tumor reduction was more than 50%. This is the first case referred to in the literature whose radiotherapy response was evaluated by magnetic resonance imaging. We describe the clinical and imaging findings of this very rare tumor and its evolution. We also comment on the literature reviewed.