Paroxysmal hemicrania (PH) is one of the trigeminal autonomic cephalgias (TACs), a group of primary headache disorders characterized by unilateral trigeminal distribution pain that occurs in association with prominent ipsilateral cranial autonomic features. The TACs are relatively rare, which is likely to be why they are poorly recognized in primary care. TACs will thus be referred to neurologists eventually, offering an excellent opportunity to diagnose and treat these patients. PH responds in a dramatic and absolute fashion to indomethacin. The importance of recognizing these syndromes is underscored by their excellent but highly selective response to treatment. This is the case report of our patient with PH and the review of current knowledge about pathophysiology of TACs, as well as differential diagnosis of other entities from this headache group.