A 67-year-old woman was referred to our hospital for persistent fever and dyspnea. Chest X-ray revealed diffuse reticulonodular shadows and high-resolution computed tomography showed randomly distributed small nodules. Examination of sputum and urine revealed acid-fast bacilli, which were later confirmed as Mycobacterium tuberculosis sensitive to all drugs. Laboratory tests revealed thrombocytopenia, an elevated concentration of fibrin degradation products, and severe hypoxemia. We therefore diagnosed her with miliary tuberculosis complicated by acute respiratory distress syndrome (ARDS) and disseminated intravascular coagulation (DIC). After admission, her status rapidly worsened and she required mechanical ventilation. Treatment with recombinant human soluble thrombomodulin (rTM) and high-dose methylprednisolone was started in addition to the antituberculosis chemotherapy. The patient's condition gradually improved and she was weaned from ventilation on day 30. She was discharged on day 92. It is generally thought that prognosis of miliary tuberculosis complicated by DIC and ARDS is very poor. A recent report suggested that rTM is effective for DIC and ARDS secondary to sepsis. This is the first report of miliary tuberculosis complicated by DIC and ARDS successfully treated with rTM.