Substernal goiter is defined as a thyroid mass of which more than half is located below the thoracic inlet. As substernal goiter enlarges within the bony thoracic cavity, and vascular and visceral structures may slowly become compressed and compromised. Airway obstruction can occur due to the compression or the displacement of the trachea. Tracheal compression may rarely lead to severe respiratory compromise. Recently, we experienced a case of huge substernal goiter causing acute respiratory failure in a 63-year old female. We present this case with a review of the literature.