Indomethacin-responsive headaches are a heterogeneous group of primary headache disorders distinguished by their swift and often absolute response to indomethacin. The epidemiology of these conditions is incompletely defined. Traditionally, indomethacin-responsive headaches include a subset of trigeminal autonomic cephalalgias (paroxysmal hemicrania and hemicrania continua), Valsalva-induced headaches (cough headache, exercise headache, and sex headache), primary stabbing headache, and hypnic headache. These headache syndromes differ in extent of response to indomethacin, clinical features, and differential diagnoses. Neuroimaging is recommended to investigate for various organic causes that may mimic these headaches. Case reports of other primary headache disorders that also respond to indomethacin, such as cluster headache, nummular headache, and ophthalmoplegic migraine, have been described. These "novel" indomethacin-responsive headaches beg the question of what headache characteristics are required to qualify a headache as an indomethacin-responsive headache. Furthermore, they challenge the concept of using a therapeutic intervention as a diagnostic criterion.