We report a 52-year-old man who had a 2-year history of right-sided occipitalgia. His pain was induced by anteroflexion of the neck. Neurologic examinations showed mild right C2 and C3 root signs. Cervical MR imaging disclosed an extra-medullary intradural crooked shadow at the level between C2 and C3 vertebrae, which was proven by selective angiogram of the ascending cervical artery to be a dural arteriovenous malformation located in the right C3 root sleeve. Embolization by an endovascular approach resulted in angiographic and clinical cure. These findings indicate that headache due to arteriovenous malformation in upper cervical region may be surgically treatable.