The clinical, epidemiological and genetic aspects of familial paragangliomas of the head and neck are discussed in relation to 6 recent cases from two families which accounted for 2 and 4 patients respectively. Tumors of the carotid body are the most frequent. Bilaterality and association with other paragangliomas are suggestive of a hereditary disorder and should lead to investigation of the family. The establishment of a family tree will define those subjects at risk in whom systemic screening should be performed. The recent advances obtained using new imaging techniques in the diagnosis, evaluation of extension and treatment should enable treatment to be instituted at an earlier stage and thus reduce functional sequelae.