The authors report on a 17-year-old boy who suffered from slowly progressiv
e and long-standing symptoms of ataxia, neck pain, and headache. Computeriz
ed tomography (CT) and magnetic resonance (MR) imaging revealed a tumor ari
sing from the floor of the fourth ventricle that resulted in a moderate hyd
rocephalus. A partial resection was performed. Histological and immunohisto
logical findings led to the diagnosis of an atypical central neurocytoma of
the fourth ventricle. The imaging features on CT scanning, MR imaging, and
proton MR spectroscopy studies, the clinical picture, and the prognosis of
this very unusual tumor are discussed. Three cases of neurocytomas in the
posterior fossa have been described to dale; however, in all three cases so
me atypical aspects were present. In the present case, with the exception o
f the very unusual location, both imaging findings and clinical history per
fectly met the definition of this rare tumor.