Patients with hereditary hemorrhagic telangiectasia (HHT) are at risk for the development of cerebral arteriovenous malformations (AVMs). The authors report a case of a spontaneously regressing cerebral AVM in a patient with HHT. The lesion was diagnosed on the basis of findings on screening magnetic resonance imaging and regressed completely before any treatment was undertaken. The spontaneous regression of cerebral AVMs is a rare and poorly understood phenomenon. Only one other instance of spontaneous regression of a cerebral AVM in a patient with HHT has been reported in the literature. The authors compare angiographic and clinical features in previously reported cases of spontaneous regression of cerebral AVMs with those in the present case to determine the characteristics common to this phenomenon.