Wegener's granulomatosis (WG) is an autoimmune disease of unknown origin characterized by necrotizing granuloma of both upper and lower respiratory tracts and glomerulonephritis. Neurological manifestations in the central nervous system (CNS) and peripheral nervous system (PNS) are a rare but well-documented feature of WG in the literature. We describe a 51-old-male who presented with complaints of slurred speech and left-sided weakness, along with bilateral pulmonary nodules on initial chest x-ray, which were associated with pulmonary symptoms in the past. A repeated computed tomography (CT) scan showed ischemia in the distribution of the right-middle cerebral artery. An open lung biopsy showed vasculitis with necrosis of a vessel wall, with a multinucleated giant cell, confirming the diagnosis of WG. The patient was successfully treated with cyclophosphamide and oral prednisone and he has continued in remission for one year.