Granulomatosis with polyangiitis is a rare disorder characterized by the necrotizing granulomatous lesions of both the upper and lower respiratory tract and glomerulonephritis. Rapidly progressive renal failure in GPA is not an uncommon presentation and always requires urgent intervention. A 25-year-old male patient who presented with fever, hematuria, hemoptysis, and decreased urine output with an abnormal renal function was found to have high titers of anti-PR3 antibody on evaluation. His prolonged hematuria followed by an anuric state during the hospital stay improved with multiple sessions of hemodialysis, and then he was started on immunosuppressive therapy with resolution of fever based on high clinical suspicion. A dramatic improvement of ongoing gross hematuria and hemoptysis was noted following a week of immunosuppression. Progression of GPA can be prevented by early diagnosis and initiation of treatment.