Microscopic polyangiitis is an exclusively small-vessel (arterioles, capillaries, or venules) vasculitis that primarily involves the kidney and often involves the lungs, skin, or nervous system. Characteristic features include focal segmental glomerulonephritis, nongranulomatous necrotizing vasculitis, and serum positive for perinuclear-staining antineutrophil cytoplasmic antibodies (P-ANCA). We report a case of microscopic polyangiitis with previously unreported eyelid and conjunctival manifestations that responded well to immunosuppressive therapy.