OBJECTIVE Eighteen patients who were judged to have systemic vasculitis (1990 American College of Reumathology criteria) affecting the kidney, from January 1988 to August 1996, were reviewed. METHOD We analyzed characteristics of clinical, biochemical, histopathological features, the interval between the onset of the symptoms to a diagnosis of disease, treatment and overall outcome. RESULTS The principal mode of presentation of the vasculitis is general, renal and pulmonary symptoms. The discovery of ANCA has improved the diagnostic procedure in patients with these diseases. Renal biopsy facilitates early diagnosis, prognosis and treatment. The renal lesion typically shows a segmental necrotizing glomerulonephritis and extracapillary proliferation forming crescent. CONCLUSIONS The systemic vasculitis with renal involvement carries a poor prognosis and high mortality. A variety of treatment has been employed, but their precise role in the management of these vasculitis is still being elucidated.