OBJECTIVE: To describe a rare case of granulomatosis with polyangiitis (GPA) in an older adult in light of the existing literature. BACKGROUND: GPA is a potentially life-threatening multisystem necrotising vasculitis, characterised by a spectrum of oral mucosal lesions that present diagnostic challenges. MATERIALS AND METHODS: A 76-year-old female patient presented with asymptomatic granular and haemorrhage swelling ("strawberry" gingivitis) affecting the upper and lower anterior and posterior gingivae. Cytoplasmic antineutrophil cytoplasmic antibody (ANCA) was positive. Concurrent kidney lesions were diagnosed. RESULTS: Histopathological examination revealed a pseudoepitheliomatous proliferation with perivascular inflammation and a dense eosinophilic infiltrate. After a 5-year follow-up, the patient showed no signs of recurrence. A literature review performed using PubMed, Web of Science and Scopus identified 22 documented cases of GPA-related oral lesions in older adults, with equal sex distribution and 63.6% occurring in the seventh decade of life. CONCLUSION: Early recognition and diagnosis of "strawberry" gingivitis in older adults with GPA are crucial, as these oral manifestations may be the initial presentation of the disease.