A sample of a primary brain tumor of glial cell origin was surgically removed from a 7-year-old girl. The histopathological analysis showed a heterogeneous tumor containing highly cellular areas composed of small, poorly differentiated cells with frequent mitoses suggestive of a glioblastoma multiforme. There were also areas presenting as features of lower-grade astrocytoma. Strong immunohistochemical staining for glial fibrillar acidic protein was demonstrated, while vimentin, neurofilament, and S-100 protein were all positive in just the astrocytic part of the tumor. The DNA extracted from a fresh tumor sample at diagnosis was processed by Southern blot analysis and hybridized with a 2.0 kb N-myc oncogene probe recognizing the first intron and the second exon of the human gene. A 20-fold amplification of the oncogene was found. The possible role of such a molecular alteration is discussed in light of the clinical presentation and histopathological features.