During a 15-year-period (1973-1987), 13 patients with familial pheochromocytoma (PCC) underwent unilateral (n = 7) or bilateral (n = 6) total adrenalectomy without major operative morbidity (1 incidental splenectomy in 8 left-side adrenalectomies) and no mortality. 1 patient died 3 years after unilateral adrenalectomy from unrelated cause. The remaining patients were followed up for a mean time of 5.2 years and could be traced and investigated. 3 patients have been adrenalectomised on the contralateral side because of PCC 5, 6 and 10 years after unilateral adrenalectomy. No patient had metastatic PCC. 1 patient sustained recurrent Addisonian crises after bilateral total adrenalectomy. 1 patient developed recurrent hypertension after bilateral adrenalectomy, but without evidence of recurrent disease. In 3 patients bilateral total adrenalectomy with adrenal cortex autotransplantation was performed, however, complete hormone supplementation therapy had to be continued because of autograft functional insufficiency. In considering the number of patients in this study, who only needed a unilateral adrenalectomy in familial PCC, the more pragmatic surgical approach to familial PCC seems to be supported. Bilateral total adrenalectomy with adrenal cortex autotransplantation was not as effective functionally as supposed by histological examination of transplant biopsy.