Mucormycosis (phycomycosis) has been reported to involve most organ systems in man. We report a young insulin-dependent diabetic who presented with acute upper airway obstruction because of isolated mucormycosis of the trachea. A combination of amphotericin B and surgical resection of the lower two thirds of the larynx and five tracheal rings with primary reanastomosis has resulted in a cure with no evidence of recurrence after nine months of follow-up. To our knowledge, this represents the first report of isolated tracheal mucormycosis and/or acute upper airway obstruction due to mucormycosis.