We report a 65-year-old caucasian male, who presented cryptogenic organizing pneumonia (COP) as first manifestation of rheumatoid arthritis. The patient started with fever, myalgias and progressive dyspnea in October 2004. The chest X-ray (CXR) and high resolution computed tomographic scan (HRCT) showed diffuse alveolar exudates with air bronchogram in both the lungs. An open lung biopsy was done and the histological image was compatible with COP. Six months later, a diagnosis of RA was made. Treatment with oral methotrexate and etanercept was prescribed with improvement in symptoms, physical examination, and laboratory tests. Even though COP after the joint involvement is found more frequently in RA, in rare cases it could be the first manifestation of this illness.