The purpose of this retrospective study is to present our approach to the management of patients with carotid body tumors (CBTs), emphasizing the role of malignancy and preoperative embolization. Between 1975 and 1998 a series of 18 patients with CBTs were treated, and 16 of them underwent successful excision of the tumor. According to the Shamblin classification, six of the tumors were type I, six type II, and six type III. In three of these patients (two with type II tumors and one with type III) in whom preoperative embolization had been performed, mean intraoperative blood loss was 400 ml, whereas in the remaining 13 cases this loss was 700 ml. Two patients with intracranial tumor spread underwent only radiotherapy. Neither postoperative deaths nor strokes occurred. Temporary cranial nerve injury occurred in four cases (25%). Local lymph node invasion was found in two patients, establishing the diagnosis of malignancy. One of these patients developed distal metastases 3 years after the operation and was treated with radiotherapy and octreotide. Follow-up ranging from 30 months to 23 years (mean 5 years) revealed no local recurrence except for the two patients who were treated with radiotherapy only. In conclusion, surgical excision remains the treatment of choice for CBTs and can be performed without major risks and with low morbidity and mortality. Preoperative embolization is helpful by diminishing intraoperative bleeding, and malignancy, though rare justifies early management.