Trigeminal autonomic cephalalgias (TAC) are primary headache disorders that are characterized by severe unilateral pain along the distribution of the trigeminal nerve with corresponding activation of the autonomic nervous system. The clinical characteristics and presentation of TAC are unique; however, there may be an overlap of these features with other painful conditions affecting the orofacial region, which can be a diagnostic challenge for the clinician. This article reports a case history and discusses the differences between clinical characteristics of TAC and other painful orofacial conditions. Refractory pain conditions and the occurrence of episodic pain attacks with accompanying autonomic symptoms necessitate a thorough evaluation to rule out rare causes of head and face pain. Schlagwörter: cephalalgias, facial pain, headache, primary headache disorders, trigeminal autonomic cephalalgias