We describe a 9-month-old boy in whom a hepatic tumor called angioma was diagnosed on ultrasonography, performed for abdominal pain. He was asymptomatic until 9 months later, when he presented with weight loss, jaundice, and a hard tumor in the left liver lobe. Radiological examination showed a calcified and heterogeneous tumor, amputation of the left portal vein, and dilatation of bile ducts, strongly suggesting malignancy. However, liver biopsy showed the typical findings of inflammatory pseudotumor, which are myofibroblastic cells and perivascular plasmocytes in a dense collagenous stroma. The tumor was surgically removed without local recurrence with a 2-year follow-up. Inflammatory pseudotumor in childhood is more common in the lung and elsewhere is often mistaken for a slowly growing cancer. This case points out the difficulties in the radiological evaluation of liver tumors in childhood and the importance of the histological differentiation of this lesion from malignancy before laparotomy for adequate indication of the treatment, which includes no medical treatment and surgery as conservative as possible.