Objective: This is a report of 2 hypertensive siblings with a history of carotid body tumors and subsequent benign adrenal pheochromocytomas (pheos) in a family where the mother had died of possible adrenal carcinoma. Clinical Presentation and Intervention: The first case was a 35-year-old woman with paroxysmal hypertensive attacks and a right adrenal mass. She had earlier undergone surgery to remove bilateral carotid body tumors. Investigation revealed excessive excretion of catecholamines and their metabolites in the urine. Abdominal MRI and I-131-MIBG scintigraphy revealed a right adrenal tumor. Right adrenalectomy was performed. The second case, the 45-year-old brother of the first case, was found to have a left adrenal mass on abdominal MRI. Catecholamines and their metabolites in the urine were found to be increased. He had also had surgery to remove bilateral carotid body tumors of the neck. Left adrenalectomy was performed. Both siblings showed no evidence of other familial syndromes, such as multiple neoplasia Conclusion: Although the combination of familial carotid body tumors and pheo is rare, a patient who remains hypertensive after removal of a carotid body tumor deserves a careful evaluation to exclude pheo. Such tumors may be extra-adrenal or multifocal. Copyright (C) 2006 S. Karger AG, Basel.