Cervicogenic headache is a relatively common and still controversial form of headache arising from structures in the neck. The estimated prevalence of the disorder varies considerably, ranging from 0.7% to 13.8%. Cervicogenic headache is a 'side-locked' or unilateral fixed headache characterised by a non-throbbing pain that starts in the neck and spreads to the ipsilateral oculo-fronto-temporal area. In patients with this disorder, attacks or chronic fluctuating periods of neck/head pain may be provoked/worsened by sustained neck movements or stimulation of ipsilateral tender points. The pathophysiology of cervicogenic headache probably depends on the effects of various local pain-producing or eliciting factors, such as intervertebral dysfunction, cytokines and nitric oxide. Frequent coexistence of a history of head traumas suggests these also play an important role. A reliable diagnosis of cervicogenic headache can be made based on the criteria established in 1998 by the Cervicogenic Headache International Study Group. Positive response after an appropriate nerve block is an essential diagnostic feature of the disorder. Differential diagnoses of cervicogenic headache include hemicrania continua, chronic paroxysmal hemicrania, occipital neuralgia, migraine and tension headache. Various therapies have been used in the management of cervicogenic headache. These range from lowly invasive, drug-based therapies to highly invasive, surgical-based therapies. This review evaluates use of drug therapy with paracetamol and NSAIDs, infliximab and botulinum toxin type A; manual modalities and transcutaneous electrical nerve stimulation therapy; local injection of anaesthetic or corticosteroids; and invasive surgical therapies for the treatment of cervicogenic headache. A curative therapy for cervicogenic headache will not be developed until increased knowledge of the aetiology and pathophysiology of the condition becomes available. In the meantime, limited evidence suggests that therapy with repeated injections of botulinum toxin type A may be the most safe and efficacious approach. The surgical approach, which includes decompression and radiofrequency lesions of the involved nerve structures, may also provide physicians with further options for refractory cervicogenic headache patients. Unfortunately, the paucity of experimental models for cervicogenic headache and the relative lack of biomolecular markers for the condition mean much is still unclear about cervicogenic headache and the disorder remains inadequately treated.