IgA nephropathy is a renal-limited form of systemic vasculitis, and pulmonary manifestations are uncommon. An initial presentation with severe diffuse alveolar hemorrhage (DAH) or pulmonary-renal syndrome is rare and only confined to a few case reports. Herein, we present a young male admitted with acute-onset dyspnea, hemoptysis, and rapidly progressive renal failure. With an initial diagnosis of an immune-mediated pulmonary-renal syndrome, he was treated with high-dose corticosteroids and therapeutic plasmapheresis along with intensive organ support (including hemodialysis, red cell transfusion, and high-flow oxygen). After a detailed laboratory evaluation and kidney biopsy, IgA nephropathy was diagnosed. The patient continued to worsen with persistent DAH and died. IgA nephropathy-associated severe DAH or pulmonary-renal syndrome is rare but increasingly recognized. The condition is difficult to diagnose early and has no proven disease-targeted therapy.