An unusual case of carotid body tumour.

Carotid body tumours are uncommon but fascinating lesions, which arise from paraganglionic tissue at the bifurcation of the common carotid artery. We report the case history of a 40-year-old female patient who presented initially with a malignant left-sided tumour, followed by a right-sided tumour 17 years later. Although there is no known family history, it is likely, because of the bilateral tumours and the young age at presentation, that the tumour was inherited in this patient. The literature on carotid body tumours is reviewed, with particular emphasis on malignant and multiple tumours and their treatment. We discuss the recently elucidated mechanism of inheritance and its implications for affected patients and their families.