Conclusion: A high rate of infrequent presentations of giant cell arteritis were seen in the ENT department and should be anticipated as a differential diagnosis in every older patient with odynophagia with high CRP values without cause in thorough ENT examination.Objective: To describe the clinical manifestation of head and neck giant cell arteritis and to derive a diagnostic pathway covering atypical cases.Method: Single-center, retrospective analysis of cases with GCA in the head and neck region (HN-GCA) (2002–2012) to describe the clinical presentation and to derive a diagnostic pathway covering manifestations presenting to an ENT department.Results: Sixty-five patients were newly diagnosed with HN-GCA in the department of otolaryngology, ophthalmology and neurology. The most frequent symptoms were loss of vision (83%) and new onset headache (63%). Eight patients (12%) presented with infrequent manifestations, predominantly in the department of otorhinolaryngology. The most common atypical presentation (50%) was odynophagia in conjunction with high CRP values misleading to an infectious cause and delaying diagnosis. A diagnostic pathway for GCA was derived based on the ACR classification criteria and the clinical findings.