The authors present the clinical, radiological, and pathological features o
f a malignant intracerebral nerve sheath tumor that occurred in the right p
arietooccipital lobe of a 4-year-old girl. Computerized tomography scanning
and magnetic resonance imaging demonstrated a 5 x 5 x 4-m multiloculated m
ass with considerable enhancement of the irregularly shaped septa and clear
ly calcified areas within the mass. Among five cases reported in the litera
ture, this patient is the youngest and represents the first case in which t
here is radiological evidence of intratumoral calcification.