Abstract: Abstract A 53 year-old man presented with a 6-year history of a mass in the left side of hisupper neck that had been increasing in size for the past year. A MRI scan showed a mass intimately surrounding the carotid body and CT showed multiple nodules in the lungs, so he was diagnosed with malignant carotid body tumor. After compression of the carotid body, the surgeons removed the tumorand the left carotid artery and then rebuilt the carotid body. The patient recovered well after surgery.The doctors agreed that the diagnosis of malignant carotid body tumor did not depend on the histology alone but on biological behaviors, namely invasion or metastasis. A PET- CT scan verified metastatic tu-mors in the lungs, and metastatic lymph nodes were also found histologically. This type of malignancy isexceedingly rare, so the doctors involved deemed it advantageous to share the surgical method and post-operative treatments.