Granulomatosis with polyangiitis (GPA) is a disease characterized by necrotizing granulomatous inflammation of small and medium vessels, mainly in the upper respiratory tract, lungs, and kidneys. Verifying the diagnosis can be difficult due to the variety of clinical presentations, possible multiple organ damage, and atypical course. GPA patients may have nonspecific symptoms characteristic of other bronchopulmonary diseases, which often leads to diagnostic errors. Methods. The article presents an analysis of literature data on the clinical and radiological aspects of lung damage during GPA and clinical observation of a 26-year-old patient who has been under regular follow-up at the TB department for infiltrative tuberculosis without bacterial excretion and received anti-tuberculosis therapy. At the end of the main course of treatment, the patient underwent surgery. Pulmonary tuberculosis was excluded based on histological and bacteriological examinations, but pneumonia with abscesses was diagnosed. Five years after the surgery, infiltrative changes in the operated right lung were detected again during the fluorography. These findings were regarded as tuberculosis. Tuberculosis treatment was carried out with a change in chemotherapy regimens due to the lack of radiological improvement. Results. The patient was referred to the Novosibirsk TB Research Institute. The institute specialists have expressed doubts about the tuberculous etiology because of the long pulmonary disease anamnesis, the previous X-ray findings, and pathological changes in the right lung. These doubts served as the basis to repeat the pathomorphological examination, which led to the diagnosis of granulomatosis with polyangiitis. Conclusion. According to the presented clinical observation, the difficulties of timely verification of the diagnosis have been demonstrated, which is associated with the lack of vigilance among doctors regarding GPA.