SUMMARYEpendymomas are relatively uncommon and present with a spectrum of biological and clinical characteristics that make specific recommendations regarding their treatment difficult and assignment of prognostic factors controversial. The case of fourth ventricular anaplastic epednymoma in a four-year-old child is reported in which the initial presentation was deterioration of the level of consciousness secondary to acute obstructive hydrocephalus. An initial insertion of a ventriculoperitoneal shunt (V-P) to deal with the acute intracranial hypertension was done. Subsequently, sub-occipital craniectomy and sub-total resection of the tumour were performed successfully. Postoperative radiotherapy was also undertaken on the patient. The pertinent literature is reviewed. At one year follow up our patient had clinically improved with no signs of recurrence. The most important prognostic factors are tumour grade and the presence of residual tumour on post operative imaging studies. A median survival of 31 months is noted in children with infratentorial ependymomas and one year survival is quoted as 81%.

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