Immunoglobulin G4-related disease (IgG4-RD) is regarded as an immune-mediated systemic fibroinflammatory disease. Several studies have linked IgG4-RD to infections such as tuberculosis and actinomycosis. However, the coexistence of IgG4-RD and non-tuberculous mycobacterium (NTM) in a single pulmonary nodule has not been reported yet.A 76-year-old male patient presented with cough and sputum. A solitary pulmonary nodule suspicious of lung cancer was found on chest CT.Through video-assisted thoracoscopic biopsy, a diagnosis of co-existing NTM and IgG4-RD in a single nodule was made.Antibiotic treatment was applied for pneumonia developed after surgery. The patient was also supported by extracorporeal membrane oxygenation and mechanical ventilation since his pneumonia was refractory to medical treatment.The patient expired on the 60th postoperative day due to multiple organ failure.IgG4-RD can occur singularly or accompanied by other diseases. We report a solitary pulmonary nodule caused by NTM and concurrent IgG4-RD, suggesting a possible association between these 2 entities. Immunologic relations between IgG4-RD and accompanying infection should be further investigated.