Two patients with asymptomatic IgA nephropathy (IgAN) and a third patient with chronic renal failure due to IgAN died following a recent onset of dyspnea, hemoptysis, and pulmonary infiltrates. In all cases, the cause of death was respiratory failure attributed to either bronchopneumonia or pulmonary edema. However, no infectious agent was identified. In all three patients, the diagnoses of IgAN and idiopathic pulmonary hemorrhage were established at postmortem examination. Acute alveolar hemorrhage was present in two patients. All three patients had heavy alveolar hemosiderin-laden macrophages, and capillaritis was recognized in two of them. The occurrence of fatal pulmonary hemorrhage in patients with IgAN is rare. Our findings suggested an immune complex-mediated pulmonary injury that was possibly related to the systemic nature of IgAN.