SummaryThis retrospective survey describes the clinical, laboratory, radiological and pathological features of 11 patients with well-documented Wegener’s granulomatosis. Our findings are similar to those previously reported in large series. Despite treatment with cyclophosphamide and corticosteroids, 6 patients have died. It is suggested that the outcome in some of them would have been better if an appropriate therapy had been initiated earlier in the course of the disease. Likewise the renal outcome was primarily determined by the interval between the onset of the disease and the institution of treatment. Some diagnostic problems are discussed in this regard, e.g. the concept of limited forms of Wegener’s granulomatosis, the dissociation in time of the cardinal features and the lack of specificity of the radiological and laboratory data.