In this clinical article, Dr. Christopher Boes of the Mayo Clinic in Rochester, Minnesota and Drs. Manjit Matharu and Peter J Goadsby of the Institute of Neurology and the National Hospital for Neurology and Neurosurgery in London, England discuss the clinical manifestations, etiology, differential diagnosis, diagnostic evaluation, and management of cough headache. In this update, 2 recent, large prospective studies of primary cough headache patients are reviewed. Morphometric MRI studies that have shed new light on the etiology of primary cough headache are summarized, and evidence that CSF leaks may present as cough headache without an orthostatic component is presented. Although indomethacin is typically used to treat primary cough headache, the authors discuss other treatment options, including topiramate.