Systemic vasculitides are a heterogenic group of diseases characterized by inflammation and necrosis of blood vessels or perivascular region. Vasculitides may occur as a main symptom of many different underlying diseases whose classification is based on calibre of the affected vessels. The diagnosis of systemic vasculitis syndromes is based on clinical and histopathological assessment in combination with serological abnormalities, especially the presence of antineutrophil cytoplasmatic autoantibodies (ANCA). We present a case report of a 19-year-old man with the diagnosis of systemic necrotizing vasculitis in the course of systemic lupus and/or Wegener granulomatosis. The diagnosis was based on presence of clinical, serological, histopatological and x-ray abnormalities and performed according to the American College of Rheumatology criteria. The treatment regimen consisted of pulses of intravenous cyclophosphamide and methylprednisolone every 4 weeks (6 times). Currently the patient has renal insufficiency with stabile serum creatinine level, mild anaemia and hypertension.