A 65-year-old woman was admitted due to poor oral intake and a dry cough over the previous 3 months. The physical examination was remarkable for bibasilar crackles, and plain chest radiography showed reticulation in both lower lung fields. A pulmonary function test demonstrated a restrictive pattern with a reduced diffusing capacity of the lung for carbon monoxide. High resolution computed tomography showed reticulation and honey-combing in both peripheral lung zones, which was consistent with usual interstitial pneumonia pattern. Her skin showed livedo reticularis. The erythrocyte sedimentation rate and C-reactive protein level were elevated, and hematuria was noted on urinary analysis. A serologic test for auto-antibodies showed seropositivity for Myeloperoxidase-Anti-neutrophil cytoplasmic antibody (MPO-ANCA). A kidney biopsy was performed and showed focal segmental glomerulosclerosis. She was diagnosed as having pulmonary fibrosis with microscopic polyangiitis (MPA) and treated with high dose steroids. Here we report a case of pulmonary fibrosis coexistent with microscopic polyangiitis.