We present the clinical observation of a 16-month-old girl treated for a po
sterior fossa ependymoma who experienced severe and delayed visual dysfunct
ion. She was initially treated by surgery and conventional chemotherapy. Wh
en she relapsed at age 3 years, the salvage treatment combined high-dose ch
emotherapy, second surgery, and local irradiation. At age 4 years, disturbe
d gait and dysarthric speech appeared rapidly, and she became unable to rec
ognize objects and people. Computed tomography revealed bilateral calcifica
tions in the cerebellum and temporal and occipital lobes but no relapse. Th
e neuropsychologic evaluations revealed signs of visual agnosia and marked
intellectual impairment. The role of the different treatment modalities in
the pathogenesis of this unusual syndrome is discussed.