Microscopic polyangiitis (MPA) rarely affects ocular small vessels, in contrast to the pulmonary and/or renal vasculature of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis. This report focuses on a 60-year-old woman who had MPA with ocular manifestations as the initial clinical symptom, which were improved by steroid administration. This case report provides an insight for early diagnosis of eye lesions in patients with MPA to prevent the development of both ocular and life-threatening organ damage.