摘要篇首： Carotid body tumor (CBT) is a rare tumor, which is composed of chromaffin cells, groups of cells associated with the autonomous system, occurring at the carotid bifurcation. This tumor grows slowly, usually with a history of several years and seldom undergoes malignant transformation. The majority of CBT are benign, only few cases of malignant CBTs were reported in the literature.1-3 Over a period of 10 years (1993-2004), 28 patients with CBT were diagnosed and surgically treated in the Department of Oral and Maxillofacial Surgery, Ninth People's Hospital, School of Stomatology, Shanghai Jiaotong University, only one was proven both clinically and histopathologically to be malignant CBT. This paper reports this unusual patient with recurrent malignant carotid body tumor as well as cervical lymph node metastases and reviews the relevant literature.