We retrospectively reviewed a 20-year experience with 59 carotid body tumors in 55 patients examined at our institution in order to determine the long-term results of surgical resection, including the rates of distant metastasis, local recurrence, and long-term survival. Complete surgical excision was possible in 52 of the 55 patients (95%). Perioperative mortality was only 2% (1 of 59 procedures), and no operative deaths occurred during the last 10 years of the study. Survival of patients after resection of a carotid body tumor was equivalent to that for sex- and age-matched control subjects. Only one patient (2%) had development of metastatic disease during long-term follow-up. Three patients (6%) had recurrence of the carotid body tumor after complete excision. All recurrent tumors were observed in patients with multiple paragangliomas or a family history of cervical paragangliomas. Therefore, we advocate early surgical resection of all carotid body tumors in low-risk patients. Such early resection maximizes the possibility of cure and minimizes the risks of neurovascular complications associated with large and neglected tumors.