Abstract
A 42-year-old man presented with a mediastinal tumour. On histological examination, tuberculosis was evoked, but no definitive conclusion could be drawn. The patient thereafter developed throat ulcerations and rapidly progressing renal insufficiency due to necrotizing glomerulonephritis. After reviewing the histological material, it was concluded that all of these manifestations were related to Wegener's granulomatosis. The presentation of Wegener's granulomatosis as a mediastinal tumour is very rare, but this form of the disease must be recognized, as early treatment with cyclophosphamide is essential for a favourable outcome.