A 45-year-old Caucasian female was seen in the outpatient department with an 8-month history of left sided otalgia, throat pain and neck swelling and a 3-day history of amaurosis fugax. On examination she was found to have a left sided level II/III neck lump. A florid peri-vascular infiltration, involving and stenosing the left common carotid artery and carotid bifurcation was identified at CT angiogram and open biopsy. Auto-immune screening was negative. Histology confirmed a diagnosis of granulomatosis with polyangiitis. We present an unusual case with specific features never previously reported in the published literature.