Trigeminal autonomic cephalalgias (TACs) are a group of primary headache disorders characterized by unilateral distribution of trigeminal pain that occurs in association with prominent ipsilateral cranial autonomic features. TACs include cluster headache, paroxysmal hemicrania, short-lasting unilateral neuralgiform headache attacks, and hemicrania continua. The severity of these disorders has major effects on quality of life. Early diagnosis and appropriate management can thus markedly improve quality of life for the patient. Subcutaneous sumatriptan and pure oxygen inhalation are the most effective abortive therapies for acute cluster headache. Home oxygen therapy (HOT) has been covered by health insurance in Japan since 2018. Several drugs, such as verapamil and prednisolone, are effective in reducing the frequency of attacks in patients with cluster headache. Calcitonin gene-related peptide monoclonal antibodies and non-invasive vagus nerve stimulation are new treatments that have proven effective against cluster headache. Patients with paroxysmal hemicrania and hemicrania continua show absolute positive responses to indomethacin. Lamotrigine and several other anticonvulsants are effective in reducing the frequency of attacks in patients with short-lasting unilateral neuralgiform headache. K E Y W O R D S calcitonin gene-related peptide, indomethacin, monoclonal antibodies, non-invasive vagus nerve stimulation, trigeminal autonomic cephalalgias How to cite this article: Imai N. Cluster headache and trigeminal autonomic cephalalgias: Recent advances in diagnosis and management.