Carotid body tumors (CBT), are rare neoplasms arising from the chemoreceptor cells of the carotid bulb. We report a case of CBT in a 41 year old female, who presented with a painless, pulsatile, gradually progressive lateral neck swelling. Diagnosis was suspected on the basis of history, clinical and radiological examination findings and a successful surgical excision of the tumor was performed. Histopathological examination confirmed the diagnosis of CBT. We also present a brief literature about CBT in terms of its clinical presentation, evaluation and management.