We herein report our experience with patients who had nontuberculous mycobacterial lung disease (NTM disease) accompanied by organizing pneumonia (OP). Out of 98 NTM disease patients who had undergone a biopsy or surgical resection, 11 patients had OP that was revealed histologically. After excluding six patients who had OP-related diseases (idiopathic interstitial pneumonia, rheumatoid arthritis, etc.), the remaining five patients were studied. Two of them (a 73-year-old man and a 66-year-old woman) showed common clinical feature: acute-onset symptoms of cough and fever, infiltrating shadows and dramatic improvement following treatment with a corticosteroid and anti-mycobacterial therapy. Our cases demonstrate that NTM disease is sometimes accompanied by OP histologically, and some such cases show common clinical features.