Abstract: Although in the majority of patients with pheochromocytoma the tumor is localized in the adrenal, up to 26% of patients have malignant/metastatic disease. Metastatic disease should be ruled out before initial surgery is attempted. Anatomical imaging modalities (computed tomography or magnetic resonance imaging) should be done first over the adrenals, and if negative over the abdomen and if no tumor is found, then the chest and neck should be covered. Regardless of the anatomical imaging results functional imaging with [123-I]- or [131-I]-metaiodobenzylguanidine (MIBG) scintigraphy should be done to corroborate the diagnosis. Negative MIBG scans should be followed by positron emission tomography (PET) studies with specific ligands like [18-F]-dopamine. Persistently negative evaluations should be followed by PET studies with non-specific ligands such as [18-F]-deoxyglucose or somatostatin receptor scintigraphy.