Objective To sum up the diagnosis, treatment, and prevention of postoperative complications of carotid body tumor. Methods The clinical data of 27 patients (30 aneurysms) with carotid body tumor who treated in our hospital from June 2005 to June 2016 were analyzed retrospectively. Results Of the 27 patients, 24 patients had unilateral lesions and 3 patients had bilateral lesions, with a total of 30 aneurysms. Three patients received color Doppler ultrasound, 18 patients received computed tomography angiography, 3 patients received magnetic resonance angiography, and 6 patients received digital subtraction angiography. According to Shamblin classification: 10 aneurysms belonged to typeⅠ, 16 aneurysms belonged to typeⅡ, 4 aneurysms belonged to type Ⅲ. Twenty aneurysms were treated with simple stripping, 6 aneurysms were treated with tumor resection and external carotid artery resection, 2 aneurysms were resected by resection, partial carotid artery resection plus external-internal carotid artery anastomosis, 2 aneurysms were resected by resection, resection of internal carotid artery, external carotid artery, and common carotid artery, as well as internal carotid artery-common carotid artery bypass. All patients underwent a successful operation with no death. After surgery,1 patient suffered from hemiplegia, 2 patients suffered from hoarseness, and 1 patient suffered from cough. Of the 27 patients, 24 patients were followed-up from 3 months to 4 years, with a median time of 2.3 years. During the follow-up procedure, the 1 patient with hemiplegic recovered to more than three levels of muscle strength after 6 months after rehabilitation, the 2 patients with hoarseness and 1 patient with cough returned to normal after 2 months after symptomatic treatment. There was no recurrence during follow-up procedure, and no other complications occurred. Conclusion Computed tomography angiography and magnetic resonance angiography are propitious to the definitive diagnosis of carotid body tumor, and actively surgical treatment should be performed once diagnosis of carotid body tumor.