A 78-year-old man visited a nearby hospital in January 2019 because of anorexia and weight loss. Chest computed tomography (CT) scan showed ground glass opacity and centrilobular nodules; chronic lower respiratory tract infection was therefore considered. However, sputum examination and blood chemistry did not reveal a specific cause. Half a year later, chest CT scan showed spontaneous regression of lung abnormalities. Two years later, he noted a productive cough, and radiological findings on chest CT scan showed worsening of the shadow. A specimen obtained by transbronchial lung cryobiopsy (TBLC) revealed IgG4-related respiratory disease (IgG4-RRD) on pathological examination. IgG4-RRD has various radiological manifestations and as a result, diagnosis can be challenging. We should consider IgG4-RRD as a differential diagnosis when chronic lower respiratory tract infection is suspected based on clinical and imaging findings. In addition, TBLC may be a useful diagnostic approach for IgG4-RRD.