Cervical localizations of extra-adrenal paragangliomas are infrequently malignant, especially in their familial forms. An immunohistochemical study was performed on 6 cervical paragangliomas which were detected in 3 sisters at the age of 20 20. One had a lymph node metastasis. This study confirmed the diagnosis of paraganglioma with endocrine chief cells and supratentacular cells. It also enabled the hormonal contents of these cervical paraganglioma to be determined. In addition to catecholamine, these tumors may (like pheochromocytomas) produce serotonin and one or more other peptides.