Wegener's granulomatosis is an idiopathic, granulomatous disease with the potential for multiple head and neck manifestations (80 % of the patients). Sinonasal symptoms are observed in more than 60 % of the patients. Due to these facts the otorhinolaryngologist plays an essential role in the multidisciplinary team involved in establishing the diagnosis early, initiating immunosuppressive therapy and providing ongoing care. The treatment is based on medical therapy consisting of corticosteroids and immunosuppressive agents, whereas surgery is reserved for selected head and neck manifestations. By means of 3 patients presenting with distinct visual loss in consequence of orbital complications with sinonasal origin the course of disease and theoretical background are reviewed. In our patients Wegener's granulomatosis was diagnosed by histopathological examination and serological detection of ANCA, cANCA. The progression of the granulomatous process and an additional purulent inflammation in 2 cases led to a temporary amaurosis and in another case to a visual loss of 50 %. Immediate orbital decompression in combination with sufficient systemic immunosuppressive treatment relieved the compression of the optical nerve and preserved vision. We conclude that early orbital decompression either by external or endonasal approach and concomitant immunosuppression is necessary to determine or improve rapidly decreasing vision subsequent to high intraorbital pressure produced by a granulomatous process and inflammation.