Up to 54% of the patients with Wegener's granulomatosis (WG) experience neurological manifestations at some point during the course of their disease. However, the central nervous system is involved in less than 10% of the patients. There have only been 4 reported cases of subarachnoid hemorrhage complicating WG. We now report the 1st patient with a clinical picture and histopathologic features consistent with WG presenting with a subarachnoid hemorrhage. Another unusual feature of this case is that perinuclear staining rather than cytoplasmic staining antineutrophil cytoplasmic autoantibodies were positive. The literature on central nervous involvement in WG is reviewed.