Inflammatory pseudotumor (IPT) of the liver is a rare entity often mis
taken for malignancy, All children reported to date have been diagnose
d and treated surgically, whereas successful medical diagnosis and tre
atment has been reported in adults. In this report, we describe the 13
th child presenting with IPT of the liver and review the literature to
determine diagnostic means to avoid unnecessary surgery, There are no
specific presumptive clinical and laboratory findings, including tumo
r markers and imaging techniques, that distinguish IPT from other spac
e-occupying liver lesions, The negativity of malignant liver tumor mar
kers and hepatitis antigens and normal liver, renal, and other systems
are supportive of IPT, The most important diagnostic aid is to bear t
his entity in mind when a child presents with an unexplained liver mas
s, Fine-needle aspiration is the most helpful measure to confirm the d
iagnosis. Surgery should be avoided in the absence of life-threatening
complications.