There are several respiratory diseases that show chronic granulomatous inflammation for the histologic finding. Among them, sarcoidosis and tuberculosis are not easy to differentiate when the clinical and radiological features present similar patterns. The increasing incidence of nontuberculous mycobacteria pulmonary disease is making it more difficult for clinicians to arrive at a proper diagnosis. A 69 year old male patient visited our hospital with chronic cough as his chief compliant. His radiologic findings were multiple enlarged mediastinal lymphadenpathies with innumerable micronodules and multiple patch infiltrations. The spleen biopsy finding showed chronic granulomatous inflammation, and Mycobacterium avium was identified on the bronchoscopic culture. Because of these findings, we treated him with drugs for nontuberculous mycobacteria disease other than sarcoidosis. However, during the treatment, his symptoms and radiological features became aggravated. Thus, we reviewed the radiologic and pathologic findings and decided to treat him with steroid, which relieved his symptoms and improved the radiologic findings. We report here on a case of sarcoidosis that was initially misdiagnosed as nontuberculous mycobacteria pulmonary disease.