Granulomatosis with polyangiitis, formerly known as Wegener’s granulomatosis, is a systemic autoimmune disorder whose etiology and pathogenesis remain poorly understood. It is characterized by the occurrence of granulomatous vasculitis of small vessels and more often affects the upper airways, lungs, and kidneys, leading to different clinical manifestations. The oral cavity is affected in up to 16% of the cases, possibly representing the first manifestation of the disease in rare instances. It typically affects the gingival tissue as a diffuse erythematous swelling with reddish punctations, often referred to as strawberry-like gingivitis. Microscopically, granulomatosis with polyangiitis shows a dense inflammatory infiltrate containing plasma cells, lymphocytes, and histiocytes that may give rise to well or poorly formed granulomas. Affected patients demand a prompt therapeutic approach that includes the combined use of cyclophosphamide and corticosteroids as an induction therapy, followed by a maintenance phase with other immunomodulatory drugs. However, patients’ prognosis remains obscure given the therapeutic toxicity and possible secondary infections.