A 3 1/2 year old girl who developed a gradual left hemiparesis and hemianopia is reported. Neuroimaging studies demonstrated an infiltrating-like high density lesion which crossed the splenium of the corpus callosum, thought to represent a glioma. Subsequent brain biopsy established the correct diagnosis of globoid cell leukodystrophy (GCL), which suggests that the radiographic appearance of late-onset GCL may mimic that of an infiltrating glioma.