Ear, nose and throat (ENT) involvement is common and often an early manifestation in ANCA-associated vasculitides (AAV). The main ENT symptoms of granulomatosis with polyangiitis (GPA) include nasal crusting, epistaxis, chronic rhinosinusitis, bilateral serous otitis media and subglottic stenosis. In eosinophilic granulomatosis with polyangiitis (EGPA), rhinitis and chronic sinusitis with or without polyps are predominant clinical features. Clinical and endoscopic examination by an ENT specialist, often complemented by CT scan, is essential for accurately assessing lesions while the contribution of nasal mucosal biopsies is limited. In addition to systemic treatments with corticosteroids and immunosuppressants, local management is often required, ranging from nasal irrigation for all patients with rhinosinusitis symptoms to surgical repair of functional and aesthetic sequelae in the course of GPA. The importance of early diagnosis of vasculitis and the impact of ENT symptoms on the patient's quality of life require close collaboration between ENT specialist and internist.