Ear, nose, and throat (ENT) involvement is a common feature in antineutrophil cytoplasmic antibody (ANCA)associated vasculitis (AAV), particularly in granulomatosis with polyangiitis and eosinophilic granulomatosis with polyangiitis. Over the last decade, substantial advancement has been made in understanding AAV pathogenesis, classification, and treatment. Typical ENT symptoms may include sinonasal, otologic, pharyngeal, and laryngeal manifestations. The otolaryngologic symptoms of AAV sometimes might be misdiagnosed in etiology as infectious or allergic. Thus, rapid recognition and early diagnosis of AAV as the cause of the symptoms prevent the risk of irreversible organ damage. The high impact of ENT symptoms on the quality of life of AAV patients confirms the importance of their early treatment through specific local and systemic approaches. Appropriate interdisciplinary management to early recognition of AAV and initiation of treatment may reduce morbidity in these patients. The purpose of this comprehensive review is to describe the clinical, histological, and radiological findings of ENT involvement in AAV and to update their surgical and therapeutic management, with a focus also on the role of a multidisciplinary team, involving the otorhinolaryngologist.