Paragangliomas arising from the carotid body in the carotid bifurcation are termed as carotid body tumors. They are usually slow growing and asymptomatic. Considering the anatomical location, invasion or pressure on the adjacent vascular and neural tissues, the importance of early diagnosis and management is critical. In this article a case of carotid body tumor excised through transverse neck skin crease incision is presented along with literature review on the diagnosis, grading and different surgical approaches.