S u m m a r y: The authors present the case-history of a 34-year-old female patient with Wegener’s granulomatosis with an unusually high number of extrarenal complications. The disease was manifested in addition to affections of the upper and lower airways by arthralgias, a febrile condition and perforation of the cornea. Subsequently the patient suffered an acute myocardial infarction due to coronary arteritis and valvulitis of the aortal valve. Affection of the skin with maculopapulous efflorescences. Another unusual complication was massive enterorrhagia. The clinical picture of the disease comprised also renal affection, i.e. necrotizing glomerulonephritis with crescents and evidence of cANCA antibodies. When clinical and laboratory remission of the disease was achieved the course of the disease was complicated by the development of subglottic stenosis of the trachea.

        Key words: Wegener’s granulomatosis, perforation of the cornea, myocardial infarction, aortal vasculitis, subglottic stenosis of the trachea, cANCA antibodies.