Insulin-like-growth factor (IGF)-II-like immunoreactivity was examined in two carotid bodies and six extra-adrenal paragangliomas with use of monoclonal antibody against rat IGF-II, which crossreacts with human IGF-II. Chief cells but not sustentacular cells of the carotid body were positive at about 10% in one case and less than 1% in another case. Among four carotid body tumours, a possible vagal body tumour and one glomus jugulare tumour, all but the glomus jugulare tumour exhibited positive tumour cells irrespective of histological variations. The frequency of positive cells ranged from 20 to 60%. IGF-II like immunoreactivity, therefore, might be widely distributed in human extra-adrenal paraganglionic tissues and tumours, although its biological role in these cells remains to be elucidated.