Between 1968 and 1988, eight patients with carotid body tumour have been operated on at Istituto di Clinica Chirurgica at the University of Pisa. We undertook the follow-up of all these patients. Two of them presented with bilateral and familial lesions. There was no mortality or morbidity, except in one patient, who had the vagus resected because it was invaded by tumour. For investigation of familial occurrence we have found ultrasound very useful for screening.