We present a case of severe respiratory failure associated with toxic epidermal necrolysis (TEN). A 3-year-old girl was administered antibiotics (cefditoren pivoxil) and NSAID (acetaminophen) for acute upper airway infection. Two days later, erythema, erosion and bullae developed widely on her body. She was diagnosed as TEN probably caused by the antibiotics, and transferred to our hospital. Steroid pulse therapy and γ-globulin therapy were started. On the 6th day, she was admitted to our ICU because of severe acute respiratory failure. Although mechanical ventilation was started, hypoxemia and acidosis deteriorated. Nitric oxide inhalation was tried, which was not effective on her oxygenation. On the 9th day, extracorporeal lung assist (ECLA) was performed for the rest of the lungs. On the 20th day, ECLA was discontinued because of the complication of consumptive coagulopathy. Although the lung compliance appeared to be gradually improved, irreversible respiratory failure developed. She had bacterial pneumonia and recurrent pneumothorax. On the 24th day, she died of respiratory failure. The rate of pulmonary complications in TEN is about 25%. The complications may incidentally take a fatal course. Adequate respiratory care is required from the earlier stage of TEN.