Atypical teratoid/rhabdoid tumor of the central nervous system has successf
ully emerged from inclusion as a form of medulloblastoma to a distinctive e
ntity over the past decade. This event has more than nosological importance
in that the five-year survival rate for medulloblastoma is considerably be
tter than for atypical teratoid/rhabdoid tumor which is uniformly poor. The
relationship of the central nervous system neoplasm to malignant rhabdoid
tumor of the kidney, beyond sharing some histologic features and a genetic
linkage to chromosome 22, needs further exploration.