A 56 year-old woman, previously diagnosed as having sinobronchial syndrome, presenting bilateral leg muscle weakness and paralysis was admitted to our hospital in April 2005. On admission, she had dyspnea on effort and a fever of 39°C. Her serum titer of MPO-ANCA was positive together with a high titer of BPI-ANCA. Chest CT showed diffuse centrilobular small nodular shadows in both lung fields. Furthermore, a pulmonary function test showed peripheral airway involvement. We diagnosed as diffuse panbronchiolitis (DPB) based on physical examination, positive cold agglutinin reaction, sinusitis, peripheral airway involvement, and radiological finding. On the 19th hospital day, she suddenly had vision impairment. Funduscopy showed multiple white spots on her fundus of the eyes. Muscle biopsy (left quadriceps muscle) revealed that the wall of a small artery and a vein had been infiltrated mainly by lymphocytes. Thus we diagnosed as DPB with MPO-ANCA-related vasculitis. She was treated with methylprednisolone pulse therapy, followed by oral prednisolone. Vasculitis associated with chronic suppurative lung disease has occasionally been reported, suggesting a possible association with chronic bacterial infection, which plays a role in the pathogenesis of ANCA-related vasculitis.