From 1971 through 1980, 106 patients underwent operations for pheochromocytoma. Twelve patients had a pheochromocytoma as a manifestation of the multiple endocrine neoplasia type 2 syndrome. Twenty patients were found to have extra-adrenal paragangliomas. Fifteen patients (14%) had malignant tumors; eight of these tumors were encountered in the group with extra-adrenal lesions. The cumulative survival probability at 5 years was 53%. Determination of urinary metanephrines and vanillylmandelic acid were the most sensitive diagnostic aids, with accuracy rates of 95% and 89%, respectively. In recent years, the measurement of fractionated urinary and plasma catecholamine levels has greatly increased the diagnostic accuracy. Preoperative localization of pheochromocytoma has improved during the last decade. Nephrotomography, selective angiography, and venous sampling have essentially been abandoned in favor of computed tomography. With an accuracy of more than 90%, computed tomography represents the major step forward in the overall management of pheochromocytoma during the past decade.