This report describes a case of granulomatosis with polyangiitis (GPA) in which the gingival manifestation was crucial in both making an early diagnosis and possibly in deciding the approach to treatment.

A 57-year-old sailor presented to the Department of Dentistry at Ulsan University Hospital complaining of gingival swelling since approximately 2 months. He had orofacial granulomatous lesions and the specific gingival manifestation of strawberry gingivitis.

The diagnosis of GPA was made on the basis of clinical symptoms and signs, and confirmed by the presence of the anti-neutrophil cytoplasmic antibody and a positive biopsy. The patient was admitted to the hospital and subsequently placed on a disease-modifying therapy regimen that included methotrexate and prednisone.

Identification of the gingival manifestation of the disease permitted an early diagnosis and prompt therapy in a disease in which time is a crucial factor. Because of its rapid progression and potentially fatal outcome, an early diagnosis of GPA is important. Therefore, dentists should be aware of the oral signs and symptoms of such systemic diseases.