A 60-year-old male presented with complaints of hemoptysis, cough, chest pain, nasal crusting, and hematuria. He had history of diabetes for the last 20 years on orally administered antihyperglycemic agents (OHAs). He was a smoker for the past 30 years. On examination, patient had strawberry gingival hyperplasia. Auscultation revealed bilateral wheeze on all areas of chest. High-resolution computed tomography (HRCT) revealed ill-defined multifocal patchy areas of airspace consolidation in both lungs involving the upper and lower lobes. Rheumatoid arthritis (RA) factor, antinuclear antibody (ANA) enzyme-linked immunoassay (ELISA), anti-cyclic citrullinated peptide (CCP) antibodies ELISA, deoxyribonucleic acid (DNA) (double strand) antibody were negative, angiotensin-converting enzyme (ACE) was within normal limits while cytoplasmic anti-neutrophil cytoplasmic antibody (C-ANCA) was positive and perinuclear anti-neutrophil cytoplasmic antibody (P-ANCA) was negative. A diagnosis of granulomatosis with polyangiitis was made in accordance with the American College of Rheumatology Criteria for Wegener's granulomatosis. Strawberry gingival hyperplasia is a rare but pathognomonic manifestation of granulomatosis with polyangiitis (GPA); hence I present this case report.