We present the experience of Hospital das Clínicas, FMUSP, with the clinical, biochemical and topographic diagnosis of pheochromocytoma, as well as the therapeutic approaches. Although new biochemical tests, as plasma metanephrines determination, have greater sensibility in the diagnosis of this tumor, more feasible tests, such as urinary metanephrines and urinary and plasma catecholamines, still have important diagnostic value. Eventual false-negative and false-positive results can be identified with stimulation and suppression tests and drug withdrawal. Magnetic resonance imaging has the greatest sensibility for the topographic diagnosis. In the absence of absolute contraindications, treatment is always surgical, and should be preceded by clinical therapy. Precise diagnosis of this tumor is important to prevent cardiovascular events that imply high morbidity and mortality, as well as to identify other neoplastic syndromes that may be associated.