The red ear syndrome was first described by Lance in 1994. Since the initial description, approximately 60 cases have been presented in the literature. The syndrome is characterized by attacks of unilateral ear discomfort or burning during which the ear becomes red. An association with upper cervical spine disorders, primary headaches and trigeminal and glossopharyngeal neuralgia was reported. Some cases were idiopathic. We report a new case of red ear syndrome that responded to greater auricular nerve blockade. The literature on the entity is reviewed and a unifying pathophysiological hypothesis of this syndrome is suggested.