We report an 86-year-old woman who presented with organizing pneumonia (OP) with a positive anti-cyclic citrullinated peptide (anti-CCP) antibodies as the first manifestation of rheumatoid arthritis (RA). She experienced dyspnea, chest X-ray showed diffuse alveolar exudates indicated OP histologically. Although she did not present with articular symptoms initially, anti-CCP antibodies measured for differentiation of RA were positive. Eight months later, she showed representative manifestations of RA. Even though OP following joint involvement is frequent in RA, in rare cases it could be the first manifestation. This is the first case showing OP with a positive result for anti-CCP antibodies as the first manifestation of RA.