A 67-year-old female patient presented with a lung nodule one month ago which was detected by physical examination. She was diagnosed with tracheobronchopathia osteochondroplastica complicated with right lower lobe nodules by chest computed tomography, bronchoscopy, and pathological examinations of the biopsy specimens. We performed lobectomy and lymphadenectomy by video-assisted thoracoscopic surgery and small pulmonary lesions were diagnosed as pulmonary adenocarcinomas. At 36 months of follow-up, the patient was free from recurrence. In conclusion, tracheobronchopathia osteochondroplastica complicated with a pulmonary adenocarcinoma is rare, and clinicians should be aware of this possibility, particularly in patients with pulmonary malignancy and tracheal irregularities on chest imaging. Otherwise, tracheobronchopathia osteochondroplastica can be easily misdiagnosed as malignant invasion of the trachea.