Granulomatosis with polyangiitis (Wegener's disease) - systemic vasculitis, initial manifestations, the clinical picture may be present in a wide variety. This leads to difficulties in establishing a timely diagnosis. The prognosis in untreated generalized granulomatosis with polyangiitis is extremely poor. The present case report illustrates a late diagnosis of granulomatosis with polyangiitis. A 53-year-old woman was diagnosed with granulomatosis with polyangiitis only after ten months of onset of disease. Wrong diagnosis of tuberculosis of ear leads to a lot of delay in the treatment this type of vasculitis. At the time of diagnosis she had generalized form of disease presented with involvement of the eyes, upper and lower respiratory tracts, kidneys, and nervous system. Remission was achieved with methylprednisolone and cyclophosphamide but suffered a relapse shortly afterwards. Further treatment with rituximab achieved a second remission, but the patient continued to suffer from dry conjunctivitis. Symptomatic therapy in this case was ineffective. An effective pathogenic therapy for this condition was instillation of cyclosporine eye drops.