The clinical features of headache and neck pain in 14 patients with extracranial vertebral artery dissection proven by angiography or magnetic resonance imaging are reported. Pain was always located on the side of the dissected vertebral artery. Whereas eleven patients had head and posterior neck pain, the others had either only posterior neck pain, no change of a chronic pre-existing headache or no pain at all. Pain started suddenly, was of sharp quality and severe intensity, different from any previously experienced headache. Following acute onset, the time course of pain was monophasic with gradual remission of a persistent headache lasting one to three weeks. A delay between onset of head or posterior neck pain and onset of neurologic dysfunction was noted in 12 patients and was less than one day and between one day and three weeks in six each. Report of this distinct type of pain, although nonspecific as an isolated symptom, should raise suspicion of an underlying vertebral artery dissection. Early confirmation of this diagnosis and subsequent anticoagulation if dissection does not extend intracranially may help prevent vertebrobasilar ischemic deficits.