Purpose: To present an update the orofacial manifestations of granulomatosis with polyangiitis (GPA) and present a clinical case with the initial signs in the oral cavity. Materials and Methods: A bibliographic search was performed on Pubmed with the keywords 'Wegener's granulomatosis', 'etiology', 'oral manifestations', 'oral cavity', 'gingiva'. The inclusion criteria were papers published in English in the last 10 years that made reference to clinical cases with in which the oral cavity was affected. The quality of the results was assessed with 'The 2013 Care Checklist'. Results: Nineteen clinical cases were analysed. The average quality was 7.68/13 (range 5-10/13). 73.7% of patients were women, the most frequent area for the lesions was the gingiva and the most prevalent lesion was gingival hyperplasia. 68.4% of the patients had this lesion as a first sign, 21.1% as a progression and 10.5% as a recurrence. 68.4% of the lesions resolved once medical treatment was established. Conclusion: GPA is a multisystem disorder associated with considerable morbidity and mortality if not treated. Early diagnosis improves the prognosis. The first manifestation of the disease can be seen in the oral cavity. It is important that dentists recognise the oral manifestation in order to improve the prognosis. Key words: granulomatosis, polyangiiitis, Wegener's granulomatosis