A 73-year-old female with interstitial pneumonia and systemic sclerosis was admitted with bloody sputum and fever. A chest radiograph showed bilateral infiltrative shadows superimposed on interstitial pneumonia. A chest computed tomograph also showed bilateral airspace consolidations and honeycomb formation. Mechanical ventilation was required because of respiratory failure. At intubation, fiberoptic bronchoscopy revealed bilateral bronchi to be filled with bloody secretions. Her serum myeloperoxidase-anti-neutrophil cytoplasmic antibody titer was slightly elevated. Steroid pulse therapy (1000 mg/day methylprednisolone for 3 days) and cyclophosphamide pulse therapy (500 mg cyclophosphamide) followed by 60 mg/day prednisolone was introduced on the basis of a diagnosis of diffuse alveolar hemorrhage. Her respiratory condition improved and chest infiltrative shadows disappeared and weaning from mechanical ventilation was successful. However, she died of ventilation insufficiency due to progression of her chronic respiratory failure. A necropsy specimen obtained from the left lung showed fibrosis, predominantly in peripheral areas of lobules, and accumulation of hemosiderin-laden macrophages, which findings were consistent with usual interstitial pneumonia and diffuse alveolar hemorrhage. A review of the world literature revealed 4 case reports of diffuse alveolar hemorrhage associated with interstitial pneumonia and systemic sclerosis. We should be aware of this rare but serious complication in the management of interstitial pneumonia associated with systemic sclerosis.