Mycobacterium gordonae is one of the most commonly isolated nontuberculous mycobacteria (NTM) in respiratory samples. Although it seldom causes pulmonary disease and is often considered contamination or "non-pathogenic", there have been reports of true infections involving pulmonary disease, soft tissue, or disseminated disease. Currently, there are no evidence-based management guidelines for M. gordonae pulmonary disease. Treatment typically includes a combination of macrolides, fluoroquinolones, linezolid, and amikacin, based on limited data regarding susceptibility to M. gordonae. Here, we reported an 81-year-old woman presenting with significant body weight loss with an abnormal chest X-ray and high-resolution computed tomography (HRCT) showing lung clustered nodules. Further microbiological criteria for NTM pulmonary disease were met. Treatment with macrolide-based combination therapy was initiated. Clinical symptoms improved, an image showed resolution, and sputum conversion was achieved during treatment. The diagnosis of M. gordonae pulmonary disease was confirmed. The patient had nearly complete remission of lung lesions and completed treatment smoothly over 9-month period.