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