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Wegener's granulomatosis is a necrotizing inflammation of small and medium size vessels with granuloma formation. It is a very heterogeneous disease in respect of severity and clinical manifestation. While it can be a rapidly progressive disease with fatal ending, there are forms limited only to one organ. Diagnosis is supported by the positivity of anti-neutrophil cytoplasmatic antibody and the presence of the typical histological findings. Unfortunately, these examinations cannot confirm clinical suspicion relatively frequently. In addition, there may be only symptoms related to one single organ for a long time at the beginning of the disease and, therefore, one have to be aware of the clinical signs and symptoms of the different organ systems. This may allow us to make an early diagnosis and start treatment in time.