Neurologists must entertain a broad differential diagnosis when considering a patient with cavernous sinus syndrome, including neoplasm, trauma, vascular causes, inflammatory processes, and infections. We report the case of a 37-year-old woman initially diagnosed with cavernous sinus syndrome, where subsequent investigations revealed findings of Takayasu's arteritis, a large vessel vasculitis. The patient also tested positive for perinuclear antineutrophil cytoplasmic antibodies, suggesting the possibility of a vasculitic spectrum disorder although no clinical features of Wegener's granulomatosis were present. Criteria for Takayasu's arteritis and its protean neurologic manifestations are reviewed. This case highlights the spectrum of vasculitic conditions that may be associated with cavernous sinus inflammation.