A case with Wegener's granulomatosis in a young woman is described. The diagnosis was made after extirpation and histological investigation of granuloma tissue from the nasal concha and by needle renal biopsy. A successful treatment was carried out with plasmapheresis, hemodialysis and immune-suppressors, the acute paroxysm being coped as well as the developed acute renal insufficiency. After each plasmapheresis a considerable reduction of the circulating immune complexes was established. After a remission of 9 months the patient developed a stage of terminal chronic renal insufficiency and was subjected to chronic dialysis with good rehabilitation.