Wegner's granulomatosis is a clinicopathologic syndrome of unknown etiology characterized by granulomatous vasculitis of the upper and lower respiratory tracts and glomerulonephritis. During the period covered by this review several articles were published describing the clinical and pathologic features of Wegner's granulomatosis. Specifically, two large series are discussed reviewing the pulmonary manifestations of the disease and the histopathology of the head and neck disease associated with Wegner's granulomatosis. The majority of publications related to Wegner's granulomatosis concern anti-neutrophil cytoplasmic antibodies and their role in the diagnosis, management, and pathogenesis of Wegner's granulomatosis. In the period covered by this article, no new reports on the therapy of Wegner's granulomatosis were reviewed. However, two articles that address the efficacy of cyclophosphamide pulse therapy in Wegner's granulomatosis have recently been published with conflicting conclusions. The data from these articles are mentioned but will be reviewed in more detail in a subsequent article.