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Granulomatous polyangiitis (GPA) is a type of vasculitis involving medium and small arteries, typically affecting the upper and lower respiratory tract with coexisting glomerulonephritis. GPA is also characterized by necrotizing granulomatous inflammation and the presence of antineutrophil cytoplasm antibodies (ANCA). So far, various infections have lead to elevation of titers of serum ANCA, making it difficult to diagnose.We report a 50-year-old woman who was diagnosed as tuberculous lymphadenitis. During the treatment by anti-tuberculosis (TB) drugs, rapidly progressive renal failure and pleurisy had appeared with elevated titer of PR3-ANCA. Renal biopsy revealed crescentic glomerulonephritis.Renal biopsy revealed crescentic glomerulonephritis and diagnosis of GPA was made.Steroid therapy had been started with continuation of anti-TB drugs.Renal dysfunction had gradually recovered and pleurisy had disappeared with decreasing titer of PR3-ANCA.This is the first report of GPA complicated by TB infection. When we encounter a case with rapidly progressive renal failure during the TB infection, complication of GPA should be suspected as 1 of the different diagnosis.