Lack of, or suboptimal, response to antimicrobial therapy prompts further investigation. We report the case of a previously healthy adult male who presented septic, with disseminated pyogenic infection and, during his hospitalization, was diagnosed with c-antineutrophil cytoplasmic autoantibody (c-ANCA)-positive granulomatosis with polyangiitis (GPA). The gene discussed is PRTN3; the disease is granulomatosis with polyangiitis.