Abstract
Wegener's Granulomatosis (WG) in its limited form can be a challenging diagnosis. ANCA, radiological investigations and histology should be interpreted in light of clinical findings. A delay in diagnosis can have profound visual consequences. Immunosuppressive therapy and co-morbidities can mask signs of orbital sepsis. A low threshold for further exploration and biopsy should be maintained if an orbital abscess is suspected. This report describes a complex case of WG that illustrates the diagnostic and therapeutic challenges inherent in this form of orbital inflammatory disease.