Polyarteritis nodosa and microscopic polyangiitis involve small and medium-sized blood vessels. The majority of patients with microscopic polyangiitis have perinuclear antineutrophil cytoplasmic antibodies, usually antimyeloperoxidase (anti-MPO) antibodies. This report describes the first case of perinuclear antineutrophil cytoplasmic antibodies (p-ANCA) associated vasculitis presenting as, and manifesting predominantly with, cholecystitis. We review the spectrum of disease in small and medium-sized vessel arteritis of the gallbladder from localized arteritis that may not require treatment other than careful observation for the development of systemic disease to multiorgan involvement requiring aggressive immunosuppressive therapy.