There is a close relationship between headache and the visual system. Visual symptoms are prominent features of clinical syndromes such as migraine, cluster headache, and the trigeminal autonomic cephalgias. There are also strong links between headache and the visual system on the basis of genetics, molecular biology, neurophysiology, and neuroimaging. Studies of these links are leading to the development of novel therapies for a variety of headache syndromes. This review is designed to summarize the most recent literature on headache and the visual system. A particular emphasis is placed on publications of interest to clinicians.