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A myxedema coma, representing the extreme feature of hypothyroidism is rare. Despite early vigorous treatment, a myxedema coma is associated with a mortality rate as high as 60%. Herein, a case of a myxedema coma, with severe hypoventilation, is described. When the patient arrived at the emergency room, she complained of dyspnea and general weakness, and was of a drowsy mental status. 7 days after admission, she was more confused and disoriented, and respiratory insufficiency had developed. Although levothyroxine was continued and her respiration improved, she still had a confused mentality and seizure developed. Despite medication her consciousness did not improve, so was discharged in despair by her family members. The respiratory abnormality with a myxedema coma is a depressed ventilatory response to hypercapnea, resulting in a decrease in alveolar ventilation, with progressive CO2 retention. An upper airway obstruction, especially during sleep, and neuromuscular dysfunction in breathing may be shown in hypothyroidism. Therefore, a myxedema coma, accompanied by severe hypoventilation, should be intensively treated with thyroid hormone replacement therapy and mechanical ventilatory support