We report a patient with mixed connective tissue disease (MCTD) who suddenly developed pulmonary distress with bilateral pulmonary infiltrates. She died in 24 h and was found at necropsy to have had a massive alveolar hemorrhage, as well as renal necrotizing vasculitis and immune complex deposits in the glomeruli. One previous case of MCTD with pulmonary hemorrhage was found in the literature. Recognition of this complication of MCTD may help in its early diagnosis.