BACKGROUND: Mycobacterium (M) talmoniae isolated from a patient with cystic fibrosis was first described in 2017, and cases of M. talmoniae remain exceedingly rare. CASE PRESENTATION: A 51-year-old woman had respiratory symptoms for 10 years. Diffuse panbronchiolitis (DPB) was detected at the first visit at our hospital. A cavity lesion in the apex of the left lung was found, and sputum and bronchoalveolar lavage fluid were acid-fast bacillus (AFB) smear- and culture-positive besides Pseudomonas aeruginosa. M. talmoniae was finally identified, and the standard combination therapy for non-tuberculous mycobacteria (NTM) was administered for 2 y referring to the drug-susceptibility test. Thereafter, the AFB culture was negative, the wall thickness of the lung cavity was ameliorated, and oxygen saturation improved. CONCLUSIONS: We encountered a rare case of M. talmoniae with DPB, for which standard combination therapy was effective. M. talmoniae may be considered a potential pathogen of lung disease, especially in patients with bronchiectatic lesions.