Eosinophilia has long been known as a hallmark of Churg-Strauss syndrome but has rarely been reported in Wegener's granulomatosis (WG). Here we describe a patient with WG who had skin, kidney and lung involvement as well as striking peripheral eosinophilia and hyperimmunoglobulinaemia E (hyper-IgE). The patient's clinical picture was complicated by intra-alveolar haemorrhage resulting in severe anaemia and respiratory failure. The pulmonary symptoms recovered completely, but the renal involvement evolved into end-stage renal failure despite intensive immunosuppressive treatment, intravenous immunoglobulin and plasmapheresis. We suggest that the presence of eosinophilia and hyper-IgE might contribute to the development of different disease patterns in WG.