A 37-year-old man presented with gingival hyperplasia accompanied by an ulcer on the eyelid, nasal obstruction, ear discharge, and discharging nodules on the cheek and back. An evaluation for infectious diseases and leukemia was negative. During his hospital stay, he had fever and migratory joint pains develop, suggesting a diagnosis of Wegener's granulomatosis, which was confirmed by a strongly positive cytoplasmic antineutrophil cytoplasmic antibody test. He responded promptly to treatment with oral prednisolone and cyclophosphamide. In retrospect, the diagnosis could have been suspected earlier because of the distinctive red, friable, and granular appearance of the gingiva.