We report a case of a single intrahepatic pheochromocytoma in the absence of an adrenal lesion and no evidence of metastatic disease. The patient had strong clinical and biochemical evidence of a pheochromocytoma. A CT scan was abnormal but nondiagnostic for pheochromocytoma. An 123I-metaiodobenzyl guanidine (MIBG) scan was falsely negative, but an MRI scan showed a definitive hepatic abnormality. After confirmation of endocrine activity by venous sampling, the tumor was surgically removed. The patient's symptoms have resolved and her plasma catecholamine levels as well as her 24-h urine catecholamine excretion have normalized. The case shows an unusual location of an isolated pheochromocytoma and provides an example of a false negative I-123 MIBG scan.