Objective: Granulomatosis with polyangiitis is an unusual multisystemic inflammatory disease, with vasculitis of small‐ and medium‐sized vessels, with a predilection for upper lower airways and kidneys. The etiology remains unknown although it may originate from different stimuli, in genetically susceptible patients.Materials and Methods: A detailed database search was performed. The variables were demographics, localization, histopathological findings, antineutrophil cytoplasmic autoantibody, cytoplasmic (c‐ANCA) tests, treatment, and follow‐up.Results: Fifty‐two cases were identified; the mean age was 49.6 years, with a range from 6 to 87 years. It was most frequently seen in females (57.7%). The most common race was white (59.6%). The most frequent location was in the maxillary gingiva (28.8%), followed by both the upper and lower gingiva (19.2%). The most common clinical presentation was “strawberry gingivitis” (61.5%). The main symptom was pain, in 50%. Regarding the c‐ANCA test, it was positive in 71.2% of cases. The most common therapy was prednisone and cyclophosphamide, utilized in 51.9%. The average follow‐up was 23.6 months, and 88.5% of patients were still alive at follow‐up.Conclusion: The diagnosis initially was difficult to establish, an early diagnosis and treatment are mandatory. If untreated the disease can be associated with morbidity and mortality. For the oral clinician, this disease needs to be addressed in the differential diagnosis of oral lesions.