Astroblastoma is a rare type of glial tumor, usually occurring in olde
r children and young adults. It has a distinctive histological appeara
nce that is characterized by a radiating arrangement of tumor cells th
at form perivascular pseudorosettes. The authors report only the secon
d case of astroblastoma presenting in congenital form. Following subto
tal tumor resection, the infant received 10 courses of chemotherapy co
nsisting of vincristine, etoposide, and carboplatinum. Evidence is pre
sented for a tumor response to chemotherapy, a previously unreported o
bservation. The child is alive 2.5 years after diagnosis with satisfac
tory functional status. Immunohistological and ultrastructural feature
s of this tumor are presented. The discussion focuses on the biology,
natural history, and management of this unusual neoplasm.