Goiter with major respiratory compromise is uncommon but troublesome. Evaluation and treatment of this condition are controversial. Of a total of 2,908 goiters operated on over a 17-year period, 58 cases with this particular complication were studied retrospectively to define optimal management. Twenty-two patients had severe or acute dyspnea, and four of them required immediate tracheal intubation. Thirty-six patients had chronic dyspnea without cyanosis. Carcinoma was present in these two groups in 50% and 11% of patients, respectively. Results of our retrospective study are as follows: long-standing tolerance of goiter did not preclude the possibility of compressive respiratory distress or carcinoma. Optimal management of goiter with respiratory compression was obtained when surgery was delayed until satisfactory operating room conditions and adequate possibilities of interpretation of pathologic conditions were united. In case of respiratory distress, tracheal intubation allowed to abide without risks. In other patients preoperative investigations were kept to a minimum. Technical artifices facilitated the extraction of the goiter via cervicotomy without sternotomy in 92% of patients with minimal morbidity. Whenever necessary, endotracheal intubation obviated the need for tracheostomy. These data suggest preventive removal of all large or substernal goiters.