In summer 1996, a 31-year-old woman developed arthralgia, subfebrility, and papular efflorescences on the skin, clinically and histologically suspect of vasculitis, to be followed by severe lung edema and anuria, with serum creatinine up to 1182 mol/L in the autumn 1996. The administration of high dose corticosteroids, plasmapheresis and hemodialysis resulted in regression of the clinical symptoms and considerable improvement of the kidney function. Kidney biopsy revealed sclerosing extracapsular glomerulonephritis with extensive fibrocellular crescents. Thereafter, the patient felt well, however, renal insufficiency showed gradual progression, so the patient was continuously treated with hemodialysis from January 1998. Two more episodes of severe lung edema occurred at the beginning of 1998 and in the autumn 1998, with rapid symptom regression upon the administration of high dose corticosteroids. In April 1998, during the episode of staphylococcal sepsis, multiple nodose shadows of the lungs were detected, to persist asymptomatically for the next six months. Toward the end of November, nodal enlargement and disruption, with the formation of cavitations occurred. The patient's general condition deteriorated gradually, and she died from respiratory arrest in February 1999. The patient received corticosteroids during most of the disease course, and cyclophosphamide only once, during the first episode of lung edema. On autopsy, a number of cavitations were observed in the lungs, with necrotic areas of a varying size and numerous cicatrices in the rest of pulmonary parenchyma. Besides fibrosis and areas of necrosis, histology showed palisading granulomas, with erythrocytes, macrophages and siderophages within the alveoles. Apart from candidal colonization of the airways, which developed in the terminal stage of the disease, all tests for fungi, Staphylococcus aureus and Mycobacterium tuberculosis were repeatedly negative. ANCA and other immunoassays were also negative on several occasions. Differential diagnosis of multiple nodose lesions of the lungs is discussed. The authors believe the patient suffered from Wegener's granulomatosis.