Granulomatosis with polyangiitis disease is a rare vasculitis characterized by granulomatous inflammation of respiratory tracts and glomerulonephritis along with vasculitis of other organs. In this study, a 14- year-old boy was referred from ophthalmology clinic to the pediatric rheumatology ward due to drug-resistant conjunctivitis. He had a history of chronic rhinorrhea and nighttime coughing, and he was diagnosed with allergic rhinitis. Complete blood count showed leukocytosis and thrombocytosis, and the estimated sedimentation rate was elevated. Laboratory tests showed hematuria, proteinuria, and highly positive antineutrophil cytoplasmic antibody. Moreover, sinus computed tomography demonstrated pansinusitis, and spiral chest computed tomography showed multiple pulmonary nodules in both his lungs. Finally, based on renal biopsy, the patient was confirmed as a case of granulomatosis with polyangiitis. It is notable that acute or chronic conjunctivitis may be a manifestation of rheumatic diseases.