Purpose of review Little is known about most disorders classified as rare primary headaches. This review summarizes current knowledge about clinical presentation, pathophysiology, epidemiology and treatment options of rare headache disorders. Increasing public awareness on the different types of rare primary headache disorders may lead to more research and better patient identification and treatment.

Recent findings The International Headache Society updated the classification criteria for primary headache disorders adding some clinical entities and moving others to different parts of the classification.

Summary Neuroimaging should be performed in all patients suspected to suffer from rare primary headache to rule out secondary causes of headache. Indometacin might have a good therapeutic effect in some of these headache entities, but larger randomized controlled trials are missing.