The management of a substernal goiter is a problem which has challenged surgeons since its first description in 1749. While the overall incidence in the United States has decreased with the routine use of iodized salt, the development of large multinodular substernal goiters in the rest of the world is still common. In addition, even in those regions where they are less common, knowledge of their treatment is important as they can represent up to 7% of mediastinal tumors. Certainly, the majority are large, benign masses found in the superior and anterior mediastinum, although from 3 to 15% can be malignant in nature. The presenting symptoms generally relate to the compressive nature of the mass on nearby structures. Up to 90% of patients report some form of respiratory symptoms in association with these masses. Diagnostic evaluation should include chest x-ray and computed tomographic (CT) scan. Needle aspiration biopsy should be avoided due to its dangerous substernal location. The treatment is surgical, as medical therapy is generally unsuccessful. Perioperative management should include careful evaluation of the airway as the extent of compression and deviation caused by the mass can lead to a difficult intubation. The vast majority of substernal goiters can be removed via a cervical incision; occasionally sternotomy or thoracotomy is necessary. Although rare, tracheomalacia secondary to prolonged compression of the trachea by the mass needs to be watched for postoperatively. Overall, the results of surgical treatment are excellent, as morbidity and mortality are minimal and patients can expect full relief of symptoms secondary to these mediastinal masses.